Many medical institutions in Korea have recently been performing an antibody screening test as one of the essential elements of a pre-transfusion test. The Dia antigen is well known as one of the antigens with low incidence among Caucasians; however, it has been discovered with a relatively higher incidence among Mongoloid populations. The frequency of the Dia antigen among the Korean population is estimated to be 6.4-14.5%. But in Korea, a screening panel of cells from abroad without Dia positive cells has been commonly used when a patient has an unexpected antibody screening test. Here we report a case of acute hemolytic transfusion reaction due to Anti-Dia antibody. To prevent other transfusion reaction by anti-Dia antibody, addition of Dia positive cells as unexpected antibody screening test is recommended.
Lee, Seung Hwan; No, Min Young
Hemolytic transfusion reactions represent one of the most common causes of transfusion-related mortality. Although many factors influence hemolytic transfusion reactions, complement activation represents one of the most common features associated with fatality. In this paper we will focus on the role of complement in initiating and regulating hemolytic transfusion reactions and will discuss potential strategies aimed at mitigating or favorably modulating complement during incompatible red blood cell transfusions.
Stowell, Sean R.; Winkler, Anne M.; Maier, Cheryl L.; Arthur, C. Maridith; Smith, Nicole H.; Girard-Pierce, Kathryn R.; Cummings, Richard D.; Zimring, James C.; Hendrickson, Jeanne E.
Classical anaphylaxis is the most severe, and potentially fatal, type of allergic reaction, manifested by hypotension, bronchoconstriction, and vascular permeability. Similarly, a hemolytic transfusion reaction (HTR) is the most feared consequence of blood transfusion. Evidence for the existence of an alternative, IgG-mediated pathway of anaphylaxis may be relevant for explaining the pathophysiology of IgG-mediated-HTRs. The purpose of this review is to summarize the evidence for this alternative pathway of anaphylaxis and to present the hypothesis that an IgG-mediated HTR is one example of this type of anaphylaxis.
Hod, Eldad A.; Sokol, Set A.; Zimring, James C.; Spitalnik, Steven L.
Knull phenotype completely lacks all Kell system antigens. Anti-Ku antibody is seen in immunized persons with Knull phenotype by transfusion or pregnancy. It can cause a fatal hemolytic transfusion reaction. A 66-yr-old male patient with liver cirrhosis visited emergency center due to acute bleeding. The patient was at hypovolemic shock status: his blood pressure was 80/50 mmHg, pulse rate was 110/min and hemoglobin level was 4.4 g/dL. Because of the presence of antibody against high incidence antigen, we could not find any compatible blood for the patient. Nevertheless, 4 units of packed RBCs had to be transfused. Moderate hemolytic transfusion reaction was developed after transfusion. At endoscopic examination, blood was spurting from gastric cardiac varix. Endoscopic histoacryl injection was tried, and bleeding was successfully controlled. After bleeding stopped, he was managed for anemia using steroid and other medical therapy instead of transfusion. His hemoglobin level was improved to 7.7 g/dL at the time of discharge. Later he has been proved to have a Knull phenotype, which is very rare, and anti-Ku antibody. This report is the first case of anti-Ku in a Knull phenotype person in Korea, who experienced a moderate hemolytic transfusion reaction. PMID:19571622
Kang, Min Gu; Lim, Young Ae; Lee, Kee Myung
Red blood cell (RBC) transfusions should usually be given only to restore or maintain oxygen delivery to vital organs and tissues. Medical history has clearly documented the importance of blood transfusion in saving lives threatened by acute haemorrhage or severe anaemia. The availability of blood products has facilitated many surgical and medical advances, allowing the support of patients who could
S L Morley
Childhood hemolytic uremic syndrome (HUS) is most often caused by enterohemorrhagic Escherichia coli (EHEC). Due to severe hemolysis, red blood cell (RBC) transfusions are often necessary, and anemia is aggravated by low erythropoietin\\u000a (EPO) levels caused by acute renal failure. In a single center, prospective study, we randomized ten children with EHEC-positive\\u000a HUS into two therapeutic groups: one receiving EPO
Lars Pape; Thurid Ahlenstiel; Martin Kreuzer; Jens Drube; Kerstin Froede; Doris Franke; Jochen H. H. Ehrich; Marion Haubitz
Hemolytic transfusion reactions (HTRs) can produce serious and potentially life-threatening complications in sickle cell disease (SCD) patients; however, the mechanisms underlying these complications remain undetermined. We established a model of alloimmune, IgG-mediated HTRs in a well-characterized humanized murine model of SCD. HTRs induced acute vaso-occlusive crisis (VOC), resulting in shortened survival of SCD mice. Acute VOC was associated with elevated circulating inflammatory chemokine levels, including striking elevation of the levels of the neutrophil chemoattractant CXCL1. Recombinant CXCL1 administration was sufficient to induce acute VOC in SCD mice, characterized by leukocyte recruitment in venules, capture of circulating red blood cells, reduction of venular flow, and shortened survival. In contrast, blockade of the CXCL1 receptor, CXCR2, prevented HTR-elicited acute VOC and prolonged survival in SCD mice. These results indicate that CXCL1 is a key inflammatory mediator of acute VOC in SCD mice. Targeted inhibition of CXCL1 and/or CXCR2 may therefore represent a new therapeutic approach for acute VOC in SCD patients. PMID:21383500
Jang, Jung-Eun; Hod, Eldad A; Spitalnik, Steven L; Frenette, Paul S
Delayed hemolytic transfusion reactions (DHTRs) are mediated by blood group antibodies that undergo anamnestic increases following antigen reexposure. Available options for the treatment or prophylaxis of DHTRs are limited. We report the use of automated red blood cell exchange (ARE) to limit hemolysis associated with an emerging DHTR. Following transfusion of 12 red blood cell units, a family member's comments led to the discovery of a patient's history of 4 alloantibodies (anti-E, anti-c, anti-Fy(a), and anti-M). Testing revealed that all 12 units were incompatible for at least 1 antigen. Six days after transfusion, the patient developed a newly positive antibody screen and direct antiglobulin test (DAT) result. To prevent further hemolysis, ARE was performed to replace incompatible red blood cells with antigen-negative units. After ARE, the patient's DAT results were negative and he was discharged without demonstrating symptoms of hemolysis. This case illustrates the use of ARE to limit hemolysis and prevent symptoms of a DHTR. PMID:23721278
Tormey, Christopher A; Stack, Gary
The minor antigen "c" of the rhesus system, which is revealed in population both in rhesus-negative and in rhesus-positive person with 80% frequency, is considered highly immunogenic, it causes severe hemolytic complications while hemotransfusion. In pregnant women it causes the immune conflict and, as a consequence, a hemolytic disease of newborn babies, demanding the treatment, using exchanging blood transfusion (erythrocytes). For the immune conflict prevention it is obligatory to determine the rhesus phenotype in donor and recipient as well as to investigate the presence of alloisoimmune antibodies in a recipient. In hemolytic disease of newborn baby the titer of antibodies towards erythrocytes in a parturient woman blood serum is obligatory estimated with individual matching of erythrocyte-containing media for the exchanging transfusion to newborn baby. PMID:22168031
Dizik, G M; Pavliuk, R P
A 46-yr-old man developed severe hypoxemia, pulmo- nary infiltrates, and an acute decrease in his leukocyte count shortly after transfusion of fresh-frozen plasma (FFP) during recovery from cardiac surgery. Cardiogenic pulmonary edema was excluded. Granulocyte-reactive and agglutinating alloantibodies were detected in the se- rum of the fresh-frozen plasma donor. The cross-match with the patient's granulocytes revealed antibodies specific for HLA
Lukas Brander; Angelika Reil; Juergen Bux; Behrouz Mansouri Taleghani; Bruno Regli; Jukka Takala
Summary Background In recent years, pulmonary transfusion reactions have gained increasing importance as serious adverse transfusion events. Methods Review of the literature. Results Pulmonary transfusion reactions are not extremely rare and, according to hemovigilance data, important causes of transfusion-induced major morbidity and death. They can be classified as primary with predominant pulmonary injury and secondary as part of another transfusion reaction. Primary reactions include transfusion-related acute lung injury (TRALI), transfusion-associated circulatory overload (TACO) and transfusion-associated dyspnea (TAD). Secondary pulmonary reactions are often observed in the wake of hemolytic transfusion reactions, hypotensive/anaphylactic reactions, and transfusion-transmitted bacterial infections. Conclusion Knowledge and careful management of cases of pulmonary transfusion reactions are essential for correct reporting to blood services and hemovigilance systems. Careful differentiation between TRALI and TACO is important for taking adequate preventive measures.
Bux, Jurgen; Sachs, Ulrich J. H.
Febrile non-hemolytic and allergic reactions are the most common transfusion reactions, but usually do not cause significant morbidity. In an attempt to prevent these reactions, US physicians prescribe acetaminophen or diphenhydramine premedication before more than 50% of blood component transfusions. Acetaminophen and diphenhydramine are effective therapies for fever and allergy respectively, so their use in transfusion has some biologic rationale. However, these medications also have potential toxicity, particularly in ill patients, and in the studies performed to date, they have failed to prevent transfusion reactions. Whether the benefits of routine prophylaxis with acetaminophen and diphenhydramine outweigh their risks and cost requires re-examination, particularly in light of the low reaction rates reported at many institutions even when pre-medication is not prescribed.
Geiger, Terrence L.; Howard, Scott C.
Chromium-51 labeled erythrocytes (Cr-51 RBC) are suitable for the study of hematologic disorders which involve relatively slow destruction of circulating erythrocytes, taking several days to several weeks. However, Cr-51 RBC are not suitable for investigating rapid hemolytic processes which occur within a matter of a few hours due to the variable and unpredictable elution of Cr-51 from the erythrocytes during the first 24 hours or so. Imaging, which could be useful in identifying organ systems involved in the hemolytic process, cannot be performed with Cr-51 RBC because of the high dose commitment caused by the low yield of gamma rays from Cr-51 (2). A method of labeling RBC with Tc-99m, which results in a radiopharmaceutical that combines the excellent dosimetric and imaging qualities of Tc-99m with an extremely stable bond between the Tc-99m and the RBC, is reported. The successful application of this technique in providing red cell support for a cancer patient with an unusual history of intravascular hemolytic transfusion reactions is also reported.
Benedetto, A.R.; Harrison, C.R.; Blumhardt, R.; Trow, L.L.
... TRALI is thought to be the third leading cause of transfusion related death. The majority of deaths were associated with fresh frozen plasma ... More results from www.fda.gov/biologicsbloodvaccines/safetyavailability
Summary As screening for transfusion-associated infections has improved, non-infectious complications of transfusion now cause the majority of morbidity and mortality associated with transfusion in the United States. For example, transfusion-related acute lung injury, transfusion-associated circulatory overload, and hemolytic transfusion-reactions are the first, second, and third leading causes of death from transfusion respectively. These complications and others are reviewed here and several controversial methods for prevention of non-infectious complications of transfusion are discussed; universal leukoreduction of red cell units, use of male-only plasma, and restriction of red cell storage age.
Gilliss, Brian M.; Looney, Mark R.; Gropper, Michael A.
Clinical signs and laboratory test results were analyzed in 70 neonates (42 boys and 28 girls) hospitalized because of neonatal haemolytic disease who were treated with exchange transfusion and later developed septicaemia. Serological Rh-D incompatibility was diagnosed in 11 children, ABO incompatibility in 59. Signs of infection appeared between days 1 and 7 after transfusion. Pneumonic signs and diarrhoea dominated clinically in 45 newborns, skin abscesses were observed in 10, osteomyelitis in 4. Septic shock occurred in 7. Gram-negative bacteria predominated (52.85%). A significant diagnostic value of the following was found (chi2): granulocytic band forms (expressed as percentages > or = 0.10, a neutrophil index > 0.2 and toxic granulations in neutrophils. These results were obtained in the early, asymptomatic stage of infection, i.e. before the exchange transfusion was performed. The importance of the presence of risk factors, not exchange transfusion per se, is stressed. PMID:8657505
A fatal transfusion reaction due to anti-Ku in a Knull (Ko) patient is reported. The patient was transfused with 34 units of incompatible RBCs during 44 days of hospitalization. Apart from the first transfusion, all subsequent transfusions failed to raise the patient's Hb. No serum antibody was identified until he was transferred to another hospital for dialysis. A compatibility test demonstrated a weak antibody and autocontrol reacting at room temperature by a manual polybrene method. The antibody was considered to be a "cold agglutinin." A blood sample was sent to a reference laboratory where the patient was found to be Knull and the antibody was identified as anti-Ku. PMID:15373542
Lin, M; Wang, C L; Chen, F S; Ho, L H
were transfused into non-Tg recipients passively immunized with monoclonal an- tibodies (Mabs). Only transfusions of in- compatible RBCs induced IgG-mediated HTRs, exemplified by rapid clearance and hemoglobinuria. Very high plasma levels of monocyte chemoattractant protein-1 (MCP-1) and interleukin-6 (IL-6), and lower levels of tumor necrosis factor- (TNF-), were induced after incompatible transfu- sion. No significant changes in IL-10, IL-12, or
Eldad A. Hod; Chantel M. Cadwell; Justine S. Liepkalns; James C. Zimring; Set A. Sokol; David A. Schirmer; Jeffrey Jhang; Steven L. Spitalnik
Five monkeys were infused with isologous, isoimmune IgG of high hemolytic titer, and five control monkeys received isologous or autologous nonimmune IgG. The former experienced severe intravascular hemolysis, manifested by a fall in hematocrit and a rise ...
C. E. Bell H. Lopas N. I. Birndorf R. W. Colman S. J. Robboy
Background To report a case of disseminated fusariosis with endogenous endophthalmitis in a patient with acute lymphoblastic leukemia. Transfusion-associated immune modulation secondary to platelet transfusion could play an important role in the pathophysiology of this case. Case Presentation A 9 year-old male with acute lymphoblastic leukemia complicated by pancytopenia and disseminated Intravascular coagulation was given platelet transfusion. He developed disseminated fusariosis and was referred to the ophthalmology team for right endogenous endophthalmitis. The infection was controlled with aggressive systemic and intravitreal antifungals. Conclusion Patients with acute lymphoblastic leukemia are predisposed to endogenous fungal endophthalmitis. Transfusion-associated immune modulation may further increase host susceptibility to such opportunistic infections.
Three decades ago, transfusion-related acute lung injury (TRALI) was considered a rare complication of transfusion medicine. Nowadays, the US Food and Drug Administration acknowledge the syndrome as the leading cause of transfusion-related mortality. Understanding of the pathogenesis of TRALI has resulted in the design of preventive strategies from a blood-bank perspective. A major breakthrough in efforts to reduce the incidence of TRALI has been to exclude female donors of products with high plasma volume, resulting in a decrease of roughly two-thirds in incidence. However, this strategy has not completely eradicated the complication. In the past few years, research has identified patient-related risk factors for the onset of TRALI, which have empowered physicians to take an individualised approach to patients who need transfusion. PMID:23642914
Vlaar, Alexander P J; Juffermans, Nicole P
It appears that delayed hemolytic transfusion reactions may occur several days after the administration of donor red cells is true even though they have been shown to be compatible in cross match tests by the antiglobulin technique. A specific case was observed in our center, which confirms the fact. The patient was a 37-year-old male suffering from intermediate ?-thalassemia. He had a history of two previous transfusions, with unknown transfusion reaction. In the last transfusion, laboratory data showed: Hb 7.8 g/dL and Hematocrit (Hct) 24.2%. The patient received two units of cross matched, compatible concentrated red blood cells (RBCs). After eight days a severe reaction was observed with clinical evidence of tachycardia, fatigue, fever, back pain, chest discomfort, jaundice, nausea and anorexia. Accordingly delayed hemolytic transfusion reaction was suspected, and anti-RBC antibodies were tested. Laboratory tests revealed the presence of three alloantibodies: Anti-N, anti-S, anti-K, and a monospecific autoanti-JKb.
Dolatkhah, Roya; Esfahani, Ali; Torabi, Seyed Esmaeil; Kermani, Iraj Asvadi; Sanaat, Zohreh; Ziaei, Jamal Eivazei; Nikanfar, Alireza; Chavoshi, Seyed Hadi; Ghoreishi, Zohreh; Kermani, Atabak Asvadi
It appears that delayed hemolytic transfusion reactions may occur several days after the administration of donor red cells is true even though they have been shown to be compatible in cross match tests by the antiglobulin technique. A specific case was observed in our center, which confirms the fact. The patient was a 37-year-old male suffering from intermediate ?-thalassemia. He had a history of two previous transfusions, with unknown transfusion reaction. In the last transfusion, laboratory data showed: Hb 7.8 g/dL and Hematocrit (Hct) 24.2%. The patient received two units of cross matched, compatible concentrated red blood cells (RBCs). After eight days a severe reaction was observed with clinical evidence of tachycardia, fatigue, fever, back pain, chest discomfort, jaundice, nausea and anorexia. Accordingly delayed hemolytic transfusion reaction was suspected, and anti-RBC antibodies were tested. Laboratory tests revealed the presence of three alloantibodies: Anti-N, anti-S, anti-K, and a monospecific autoanti-JK(b). PMID:24014947
Dolatkhah, Roya; Esfahani, Ali; Torabi, Seyed Esmaeil; Kermani, Iraj Asvadi; Sanaat, Zohreh; Ziaei, Jamal Eivazei; Nikanfar, Alireza; Chavoshi, Seyed Hadi; Ghoreishi, Zohreh; Kermani, Atabak Asvadi
Q fever is a worldwide zoonotic infection that caused by Coxiella burnetii, a strict intracellular bacterium. It may be manifested by some of the autoimmune events and is classified into acute and chronic forms. The most frequent clinical manifestation of acute form is a self-limited febrile illness which is associated with severe headache, muscle ache, arthralgia and cough. Meningoencephalitis, thyroiditis, pericarditis, myocarditis, mesenteric lymphadenopathy, hemolytic anemia, and nephritis are rare manifestations. Here we present a case of acute Q fever together with Coombs' positive autoimmune hemolytic anemia (AIHA) and tubulointerstitial nephritis treated with chlarithromycin, steroids and hemodialysis. Clinicians should be aware of such rare manifestations of the disease. PMID:22607576
Korkmaz, Serdal; Elaldi, Nazif; Kayatas, Mansur; Sencan, Mehmet; Yildiz, Esin
The beginning of the modern era of blood transfusion coincided with World War II and the resultant need for massive blood replacement. Soon thereafter, the hazards of transfusion, particularly hepatitis and hemolytic transfusion reactions, became increasingly evident. The past half century has seen the near eradication of transfusion-associated hepatitis as well as the emergence of multiple new pathogens, most notably HIV. Specific donor screening assays and other interventions have minimized, but not eliminated, infectious disease transmission. Other transfusion hazards persist, including human error resulting in the inadvertent transfusion of incompatible blood, acute and delayed transfusion reactions, transfusion-related acute lung injury (TRALI), transfusion-associated graft-versus-host disease (TA-GVHD), and transfusion-induced immunomodulation. These infectious and noninfectious hazards are reviewed briefly in the context of their historical evolution.
Klein, Harvey G.
Summary The majority of acute infection-associated hemolytic diseases of infancy and childhood have been suggested to be caused by exogenic alterations of the erythrocyte surface, though laboratory methods for their further evaluation were not yet available. Investigating 96 children, the present study characterizes 72% of cases as corresponding to this type of acute acquired hemolytic anemia, which cannot be clearly
R.-C. Seitz; G. Buschermöhle; G. Dubberke; R. Herbrand; M. Maiwald; H. H. Hellwege
Transfusion-related acute lung injury (TRALI) is an underdiagnosed serious complication of blood transfusion characterized by the rapid onset of respiratory distress, hypoxia, and noncardiogenic pulmonary edema during or soon after blood transfusion. The presence of anti-HLA and/or antigranulocyte antibodies in the plasma of donors is implicated in the pathogenesis of TRALI. We report 2 cases of TRALI that were caused by designated blood transfusion between mothers and their daughters; one in a 4-month-old girl who received designated packed RBCs donated by her mother and the second in a 78-year-old mother who received blood from her daughter. In both cases, examination of mother's serum revealed panel-reactive cytotoxic HLA antibodies. It is most likely that the mothers were sensitized from earlier pregnancy and produced HLA antibodies against the daughters' paternally derived HLA antigens. Designated blood transfusion between multiparous mothers and children might add an additional transfusion-related risk owing to the higher likelihood of the HLA antibody-antigen specificity between mother and child. PMID:15080312
Yang, Xu; Ahmed, Shahid; Chandrasekaran, Visalam
Fibronectin (FN) has been detected on red blood cell (RBC) surfaces using fluorescent microscopy and transmission electron microscopy following the transfusion of incompatible blood to dogs. This study was an attempt to demonstrate and quantitate RBC boun...
D. A. Ferguson
Although rare, transfusion-associated bacterial contamination (TABC) is nowadays the main risk associated with platelet concentrate (PC) transfusion. Consequences vary from spontaneously resolving symptoms to severe sepsis and death. In this report we have summarised a case of bacterial contamination and sepsis during PC transfusion in a patient with acute myeloid leukaemia. Fifteen minutes after the PC transfusion began, she developed chills and rapidly worsened to septic shock. The episode was managed appropriately. The patient's blood cultures and PC unit cultures grew Escherichia coli. The microbiological susceptibilities of isolates from the patient and platelet bag were identical. No other source of E coli was found. Donor and blood products issued from the same donation investigations were negative. The causality between sepsis and PC transfusion might be difficult to confirm. As no method is available in daily practice to eliminate TABC risk, physicians should always consider TABC by immediately stopping the transfusion and conducting appropriate investigations. PMID:24172770
Haesebaert, Julie; Bénet, Thomas; Michallet, Mauricette; Vanhems, Philippe
Introduction In this study, we sought to determine the association between red blood cell (RBC) transfusion and outcomes in patients with acute lung injury (ALI), sepsis and shock. Methods We performed a secondary analysis of new-onset ALI patients enrolled in the Acute Respiratory Distress Syndrome Network Fluid and Catheter Treatment Trial (2000 to 2005) who had a documented ALI risk factor of sepsis or pneumonia and met shock criteria (mean arterial pressure (MAP) < 60 mmHg or vasopressor use) within 24 hours of randomization. Using multivariable logistic regression, we examined the association between RBC transfusion and 28-day mortality after adjustment for age, sex, race, randomization arm and Acute Physiology and Chronic Health Evaluation III score. Secondary end points included 90-day mortality and ventilator-free days (VFDs). Finally, we examined these end points among the subset of subjects meeting prespecified transfusion criteria defined by five simultaneous indicators: hemoglobin < 10.2 g/dL, central or mixed venous oxygen saturation < 70%, central venous pressure ? 8 mmHg, MAP ? 65 mmHg, and vasopressor use. Results We identified 285 subjects with ALI, sepsis, shock and transfusion data. Of these, 85 also met the above prespecified transfusion criteria. Fifty-three (19%) of the two hundred eighty-five subjects with shock and twenty (24%) of the subset meeting the transfusion criteria received RBC transfusion within twenty-four hours of randomization. We found no independent association between RBC transfusion and 28-day mortality (odds ratio = 1.49, 95% CI (95% confidence interval) = 0.77 to 2.90; P = 0.23) or VFDs (mean difference = -0.35, 95% CI = -4.03 to 3.32; P = 0.85). Likewise, 90-day mortality and VFDs did not differ by transfusion status. Among the subset of patients meeting the transfusion criteria, we found no independent association between transfusion and mortality or VFDs. Conclusions In patients with new-onset ALI, sepsis and shock, we found no independent association between RBC transfusion and mortality or VFDs. The physiological criteria did not identify patients more likely to be transfused or to benefit from transfusion.
PurposeAlthough transfusion has been linked to the development of atrial fibrillation (AF) in cardiac surgical patients, this association has not been investigated in patients with acute myocardial infarction (AMI). Evidence supports an inflammatory mechanism in the development of AF, and red cell transfusions also elicit an inflammatory response. We therefore sought to evaluate whether packed red blood cell transfusion increases
M. Kamran Athar; Sidharth Bagga; Nanda Nair; Vivek Punjabi; Karen Vito; Christa Schorr; David R. Gerber
Transfusion-related acute lung injury (TRALI) is the major cause of transfusion related morbidity and mortality, world wide. Efforts to reduce or eliminate this serious complication of blood transfusion are hampered by an incomplete understanding of its pathogenesis. Currently, TRALI is thought to be mediated by donor alloantibodies directed against host leukocytes or the result of two distinct clinical events. For both proposed mechanisms the neutrophil (PMN) is the key effector cell. This paper reviews TRALI pathophysiology, explores the role of the PMN, details practical information for appropriate diagnosis, and promotes further studies into the pathogenesis of TRALI.
Fung, Y.L.; Silliman, C.C.
Objective: To study the effects of transfusion on the clinical course and oxygenation indexes of children with sickle cell disease and acute chest syndrome. Methods: During a 2-year period, 36 children with sickle cell disease admitted with a total of 40 episodes of acute chest syndrome were examined. Patients were given a clinical severity score indicative of the degree of
Umit Emre; Scott T. Miller; Manuel Gutierez; Phillip Steiner; Sreedhar P. Rao; Madu Rao
Splenic rupture is a very rare event in adult homozygous sickle cell patients. The authors describe a 19-year-old patient with homozygous sickle cell disease who experienced an acute splenic rupture crisis requiring emergent splenectomy. He had been receiving chronic blood transfusions regularly for 7 years secondary to a previous stroke. It is possible that these transfusions contributed to regeneration of splenic red pulp, which allowed a crisis to occur at an advanced age. PMID:15591912
Shoemaker, Michael T; Pitney, Aaron C; Harford, David J; Barker, James A
Background Red cell transfusion is associated with lung injury in susceptible hosts, although many cases do not meet criteria for transfusion related acute lung injury. Patients with underlying pulmonary fibrosis can exhibit precipitous deteriorations in respiratory status of unknown etiology defined as acute exacerbations due to superimposed lung injury syndrome. It is unclear whether red cell transfusion is associated with acute exacerbation of underlying pulmonary fibrosis. Case Report We describe a patient who underwent an uneventful elective left total hip replacement but developed anemia post-operatively. Twenty-four hours following transfusion of her fifth non-leukoreduced AS-5 red cell unit, she developed new bilateral airspace infiltrates associated with progressive hypoxemia. These RBC units were 35-38 days old. Despite supportive care and diuresis, patient remained profoundly hypoxemic with infiltrates that progressed to fibrosis. Results The patient had mild sub-clinical lower-lobe predominant interstitial pulmonary fibrosis but developed diffuse bilateral ground glass opacities with areas of consolidation 24 h after receiving her last RBC unit. Transbronchial biopsy of the right lower lobe showed active organizing pneumonia and underlying interstitial fibrosis, supporting the clinical diagnosis of acute exacerbation of pulmonary fibrosis. The bronchoalveolar lavage showed progressive bloody effluent, consistent with diffuse alveolar hemorrhage, a marker of lung injury. There was no evidence of viral inclusions, fungal elements, pneumocystis, or bacterial organisms. Conclusion Transfusion of multiple units of aged RBCs was temporally associated with an acute exacerbation and rapid progression of underlying sub-clinical pulmonary fibrosis.
Woodske, Matthew; Donahoe, Michael P.; Yazer, Mark; Lee, Janet S
Transfusion-related acute lung injury (TRALI) is a noncardiogenic pulmonary edema that occurs during or within 6 hours after transfusion. Risk factors for TRALI, which is relatively common in critically ill patients, include recent surgery, hematologic malignancy, and sepsis. Here, we report a case of TRALI induced by anti-human leukocyte antigen (anti-HLA) class II antibodies (HLA-DR) occurring after transfusion of platelet concentrates in a patient with acute leukemia. Although most patients with TRALI show improvement within 48-96 hours, our patient's condition rapidly worsened, and he did not respond to supportive treatment. TRALI is a relatively common and serious adverse transfusion reaction that requires prompt diagnosis and management.
Jin, Sun Mi; Jang, Moon Ju; Huh, Ji Young; Park, Myoung Hee; Song, Eun Young
A great variety of patient- and product-related factors influence the outcome of platelet transfusions. Our study assessed the predictive value of a flow cytometric platelet cross match test for the outcome of HLA matched and unmatched platelet transfusions in patients with acute leukemia. Thirty nine patients (26 adults and 13 children) received 60 ABO compatible apheresis platelet unites ranging from 1 to 4 per patient (mean = 1.54; median = 2). We performed flowcytometric platelet cross-matching, HLA Class I typing by sequence-specific primer (SSP) for patients and complement-dependent cytotoxicity (CDC) for donors and screening of HLA Class I antibodies by ELISA. Effectiveness of platelet transfusion was evaluated using the corrected count increment which was calculated at 60 min and 18- to 24-h posttransfusion. Multivariate analysis was performed to detect which variable can predict transfusion response more than others. Cross-matched platelet transfusions associated with good response in 51.4% of transfusion events in adults and 73.3% in children. The noncrossmatched platelet transfusions associated with poor response in 83.3% in adults and 100% in children (P-values 0.143, 0.041, respectively). In the presence of clinical factors or HLA alloimmunization in adults, cross-matched platelets were associated with good response in 29.6 and 22.2% respectively. In children this occurred in 81.8 and 66.7%, respectively. In presence or absence of HLA matching, flow cytometry platelet cross-matching was the most predictor for transfusion response (P = 0.05). Because of the difficulties to find frequent HLA matched donors for acute leukemia patients; flow cytometric platelet cross-matching may provide the most useful way for selecting donors. It is useful even in the presence of alloimunization in children. PMID:21312256
Sayed, Douaa; Bakry, Rania; El-Sharkawy, Nahla; Zahran, Asmaa; Khalaf, Muhammed R
The management model based on risk prevention has become a major influence in shaping policies for transfusion safety. There are approximately sixty interactions between the health worker and the patient during the transfusion process,representing the number of times where you have the opportunity to make a mistake.We present an analysis of the weaknesses of the National Blood System, with particular attention to the haemovigilance donor and patient. The proposals include the implementation of the National Blood containing the need to establish from the National Blood Safety, significant changes in the regulatory framework and the internal regulations of the Ministry of Health, the CNTS and COFEPRIS. Is required to promote and coordinate the collection of accurate information from the committees of transfusion medicine, which will be accompanied by an initial diagnosis from the National Survey of Blood. Requires notice to other forms of funding to ensure the viability of the projects operating blood bank. Finally, as a strategic resource, the blood is of public, so access should not be restricted. PMID:23435081
Baptista González, Héctor
Summary Background Organophosphorus compounds (OP) are a group of substances used in agriculture as pesticides and are also used as military poisoning agents (MPA). Intoxication by these agents may cause severe systemic disturbances related to both the exposure time and lethal agent concentration. Toxic effects result from an excess of the endogenous neurotransmitter, acetylcholine (ACh), because decomposition of Ach by cholinesterases is blocked by OP. Case Report The authors describe a case in which an acute OP poisoning was managed both conventionally and with cholinesterase substitution by blood transfusion. Conclusions Whole blood transfusion could be beneficial in the treatment of these life-threatening medical conditions.
Ryniak, Stan; Harbut, Piotr; GoYdzik, Waldemar; Sokolowski, Janusz; Paciorek, Przemyslaw; Halas, Joanna
ObjectiveThe safe lower limit of haematocrit or haemoglobin that should trigger a red blood cell (RBC) transfusion has not been defined. The objective of this study was to examine the physiological effects of anaemia and compare the acute responses to transfusion in preterm infants who were transfused at higher or lower haematocrit thresholds.MethodsThe authors studied 41 preterm infants with birth
Laura K Fredrickson; Edward F Bell; Gretchen A Cress; Karen J Johnson; M Bridget Zimmerman; Larry T Mahoney; John A Widness; Ronald G Strauss
Summary Transfusion-related acute lung injury (TRALI) is the most common cause of serious morbidity and mortality due to hemotherapy. Although the pathogenesis has been related to the infusion of donor antibodies into the recipient, antibody negative TRALI has been reported. Changes in transfusion practices, especially the use of male-only plasma, have decreased the number of antibody-mediated cases and deaths; however, TRALI still occurs. The neutrophil appears to be the effector cell in TRALI and the pathophysiology is centered on neutrophil-mediated endothelial cell cytotoxicity resulting in capillary leak and ALI. This review will detail the pathophysiology of TRALI including recent pre-clinical data, provide insight into newer areas of research, and critically assess current practices to decrease it prevalence and to make transfusion safer.
Silliman, Christopher C; Fung, Yoke Lin; Ball, J Bradley; Khan, Samina Y
In association with atypical pneumonia, a patient developed acute severe autoimmune hemolytic anemia. Hemoglobin temporarily was only 7.0 g\\/100 ml, so that the patient needed red blood cell (RBC) transfusion. Hemolysis was found to be caused by high titer cold agglutinins (CA), which occurred transiently during the acute period of the disease. CA of two different specificities, anti-I and anti-Fl,
A. L. König; H. Kather; D. Roelcke
TRAGI (transfusion-related acute gut injury) is an acronym we proposed to characterize a severe neonatal gastrointestinal reaction temporally related to a transfusion of packed blood red cells (PRBCs) for anemia in very low birth weights. The following are in support of a causative relationship: (1) the timing of necrotizing enterocolitis after a PRBC transfusion not being random, (2) traditional risk factors for necrotizing enterocolitis are often absent, (3) significant anemia appears to be a universal finding, (4) the age of donor blood is often slightly older than controls, (5) TRAGI is not postnatal age dependent, and (6) TRAGI does not show a centering at 31 weeks' postconceptual age as does nontransfusion-related NEC. Although TRAGI is linked to the timing of PRBC transfusions, we propose a novel hypothesis that the convergence at 31 weeks' postconceptual age for classic NEC approximates the age of presentation of other oxygen delivery and neovascularization syndromes (eg, retinopathy of prematurity), suggesting its etiologic link to a generalized systemic maturational mechanism or another common developmental theme. This report will begin by reviewing the history of the clinical presentation and discovery of TRAGI and will then analyze various pathophysiologic mechanisms that may account for the phenomenon when clinicians render therapies. We will end by a call to action for randomized clinical trials to test various etiologic theories. PMID:22818551
La Gamma, Edmund F; Blau, Jonathan
Anemia during cardiopulmonary bypass (CPB) is strongly associated with acute kidney injury in clinical studies; however, reversal of anemia with red blood cell (RBC) transfusions is associated with further renal injury. To understand this paradox, we evaluated the effects of reversal of anemia during CPB with allogenic RBC transfusion in a novel large-animal model of post-cardiac surgery acute kidney injury with significant homology to that observed in cardiac surgery patients. Adult pigs undergoing general anesthesia were allocated to a Sham procedure, CPB alone, Sham+RBC transfusion, or CPB+RBC transfusion, with recovery and reassessment at 24 h. CPB was associated with dilutional anemia and caused acute kidney injury in swine characterized by renal endothelial dysfunction, loss of nitric oxide (NO) bioavailability, vasoconstriction, medullary hypoxia, cortical ATP depletion, glomerular sequestration of activated platelets and inflammatory cells, and proximal tubule epithelial cell stress. RBC transfusion in the absence of CPB also resulted in renal injury. This was characterized by endothelial injury, microvascular endothelial dysfunction, platelet activation, and equivalent cortical tubular epithelial phenotypic changes to those observed in CPB pigs, but occurred in the absence of severe intrarenal vasoconstriction, ATP depletion, or reductions in creatinine clearance. In contrast, reversal of anemia during CPB with RBC transfusion prevented the reductions in creatinine clearance, loss of NO bioavailability, platelet activation, inflammation, and epithelial cell injury attributable to CPB although it did not prevent the development of significant intrarenal vasoconstriction and endothelial dysfunction. In conclusion, contrary to the findings of observational studies in cardiac surgery, RBC transfusion during CPB protects pigs against acute kidney injury. Our study underlines the need for translational research into indications for transfusion and prevention strategies for acute kidney injury.
Patel, Nishith N.; Lin, Hua; Toth, Tibor; Welsh, Gavin I.; Jones, Ceri; Ray, Paramita; Satchell, Simon C.; Sleeman, Philippa; Angelini, Gianni D.
Transfusion-related acute lung injury (TRALI) has been the leading cause of transfusion-associated death for nearly a decade. Recent TRALI mitigation strategies focused on reduction of leukocyte antibodies in high volume plasma products appear to be successful in reducing TRALI events and deaths, but additional preventive measures are needed. Future possibilities include, screening of donors for neutrophil antibodies, processing of blood products to reduce or remove biologic response modifiers, and the more judicious use of blood. There are currently no specific TRALI therapies. The pathogenesis of TRALI and acute lung injury-acute respiratory distress syndrome (ALI-ARDS) is quite similar; both involving interactions of activated platelets, neutrophils, and pulmonary endothelium resulting in lung damage, capillary leak, and pulmonary edema. Greater understanding of these interactions and the key molecules involved will lead to development of potential new targets for therapy. In this review, future possible preventive measures to further reduce the occurrence of TRALI will be discussed, including TRALI caused by biologic response modifiers (BRMs), like bioactive lipids and sCD40L, which are not addressed by current preventive actions that only target leukocyte antibodies in high-volume plasma products. Insights already gained from studies of ALI-ARDS treatments will be summarized and discussed as possible therapeutic targets for treatment of patients experiencing TRALI. PMID:22621267
Curtis, Brian R
Transfusion-related acute lung injury (TRALI) is a severe pulmonary complication following blood transfusions. We experienced a case of possible TRALI during the course of EBV-associated hemophagocytic lymphohistiocytosis (EBV-HLH). A 19-year-old woman was admitted to our hospital suffering from fever and abdominal pain. Her laboratory data revealed pancytopenia, liver damage, coagulopathy, and a high titer of EBV-DNA. Computed tomography showed hepatosplenomegaly and bone marrow aspiration revealed hemophagocytosis and the proliferation of atypical lymphocytes. A diagnosis of EBV-HLH was made and plasma exchange was performed. Severe hypoxia due to pulmonary edema developed two hours after starting the plasma transfusion. Methylprednisolone pulse therapy and non-invasive positive pressure ventilation ameliorated her respiratory condition. Anti-HLA class I and II antibodies were detected in donor sera and a cross-match test between patient lymphocytes and donor plasma was positive. To the best of our knowledge, this is the first case report of TRALI complicated with EBV-HLH. It is possible that hypercytokinemia accompanied by HLH was associated with the onset of TRALI. PMID:23666220
Nakashima, Eriko; Shiratsuchi, Motoaki; Honda, Emi; Fujioka, Eriko; Ohno, Hirofumi; Nakashima, Yasuhiro; Matsushima, Takamitsu; Iwasaki, Hiromi; Abe, Yasunobu; Takayanagi, Ryoichi
BACKGROUND Packed Red Blood Cell (PRBC) transfusion is associated with Acute Lung Injury (ALI) development after trauma, but this risk may not be constant through time after trauma. We hypothesized the relationship between PRBC delivery and ALI risk varies through time after injury. METHODS Data were collected prospectively from 1999–2006. Inclusion criteria: age > 13 years, SICU admission, and injury severity score (ISS) ? 16. Exclusion criteria included discharge/death within 24 hours of admission. Patients were followed prospectively for ALI development for 5 days after trauma. Discrete time models were fit to test the association of timing of PRBC delivery with development of ALI while controlling for patient demographics, resuscitation variables, ISS, and APACHE III scores. RESULTS At total of 602 patients were included. Median age was 33 years, 77% were male, and 50% were African American. Using a discrete time-survival model, the relation between transfusion and ALI development was found to vary by transfusion time-window (p<0.0001). The major effect of PRBC delivery on ALI risk occurred in the first 24 hours after trauma; this finding persisted in multivariable modeling (adjusted OR = 1.07 per unit; 95%CI 1.02–1.11, p<0.001). Cumulative incidence of ALI approached 50% in patients receiving ? 6u PRBC in the first 24 hours. CONCLUSIONS The association between PRBC transfusion and ALI development in trauma patients is time-dependent, with PRBC delivery in the first 24 hours after injury driving the overall relation. Each PRBC unit during this time period increases odds of subsequent ALI development by 7%.
Holena, Daniel N; Netzer, Giora; Localio, Russell; Gallop, Robert J; Bellamy, Scarlett L; Meyer, Nuala J; Shashaty, Michael GS; Lanken, Paul N; Kaplan, Sandra; Reilly, Patrick M; Christie, Jason D
Background Platelet transfusion is universally employed in acute leukemia. Platelet concentrate supernatants contain high concentrations of biologic mediators that might impair immunity. We investigated whether washed platelet and red cell transfusions could improve clinical outcomes in adult patients with acute leukemia. Methods A pilot randomized trial of washed, leukoreduced ABO identical transfusions versus leukoreduced ABO identical transfusions was conducted in 43 adult patients with acute myeloid or lymphoid leukemia during 1991–94. Primary endpoints to be evaluated were platelet transfusion refractoriness, infectious and bleeding complications and overall survival. Results There were no significant differences in infectious or major bleeding complications and only one patient required HLA matched platelet transfusions. Minor bleeding was more frequent in the washed, leukoreduced arm of the study. Confirmed transfusion reactions were more frequent in the leukoreduced arm of the study. Overall survival was superior in the washed arm of the study (40% versus 22% at 5 years), but this difference was not statistically significant (p = 0.36). A planned subset analysis of those ?50 years of age found that those in the washed, leukoreduced arm (n = 12) had a 75% survival at five years compared with 30% in the leukoreduced arm (n = 10) (p = 0.037) Conclusion This study provides the first evidence concerning the safety and efficacy of washed platelets, and also raises the possibility of improved survival. We speculate that transfusion of stored red cell and platelet supernatant may compromise treatment, particularly in younger patients with curable disease. Larger trials will be needed to assess this hypothesis.
Blumberg, Neil; Heal, Joanna M; Rowe, Jacob M
Previously reported cases of acute generalized exanthematous pustulosis secondary to brown recluse spider bite have been questioned due to lack of identification of the spider or because of the concomitant administration of antibiotics. We report a 9-year-old boy who arrived at the emergency department with a confirmed Loxosceles reclusa bite to the neck. On the third day of hospitalization, he developed hundreds of monomorphous, sterile pustules, initially in intertriginous areas. The eruption disseminated and was followed by pinpoint desquamation typical for acute generalized exanthematous pustulosis. During this he also developed late onset Coombs-positive hemolytic anemia and systemic loxoscelism. Sphingomyelinase in Loxosceles venom induces the production of interleukin-8 and granulocyte-macrophage colony-stimulating factor, cytokines involved in the pathogenesis of acute generalized exanthematous pustulosis, providing a mechanism by which Loxosceles reclusa bite may trigger acute generalized exanthematous pustulosis. We suggest that this case adds Loxosceles envenomation to the spectrum of agents that can trigger acute generalized exanthematous pustulosis. PMID:22082464
Lane, Leanna; McCoppin, Holly H; Dyer, Jonathan
The perinatal morbidity and mortality risk in monochorionic twin pregnancies are 3-5-fold increased compared to those of dichorionic twin pregnancies. Partially, this is due to the higher rate of preterm delivery but also to the twin-to-twin transfusion syndrome (TTTS). Caused by unidirectional blood flow via placental anastomoses, the TTTS leads to weight differences of more than 20% between monochorial twins. The blood donor often shows oligohydramnios, whereas the recipient shows polyhydramnios. Lewi et al. demonstrated, in a study with 202 monochorionic twin pregnancies, a 9% rate of severe TTTS. The mortality of this complication is about 90% when untreated. In contrast to the chronic TTTS, little is known about the acute intrapartal one, which is characterised by anaemia and hypovolaemia of the donor and polyglobulia of the recipient without significant weight differences between the two. In most cases, anaemia occurred after normal delivery of the first twin. Still, there are no means or signs for early detection. We describe the case of a 30-year-old primigravida with a monochorionic diamniotic twin pregnancy. During pregnancy, no evidence of TTTS could be detected. At 37 + 1 weeks gestation labour was induced with prostaglandin-containing gel. Both foetuses showed cephalic presentation. The CTG of the first twin showed a conspicuous heart rate. After labour the first twin presented with anaemia and hypovolaemic shock, the APGAR was 2/7/8. The infant's haemoglobin was 13.7 g/dL. After delivery, the second twin with APGAR 10/10/10 showed a haemoglobin of 19.6 g/dL, which is in the upper normal range. Their birth weights differed by merely 10.4%. Acute TTTS is frequently characterised by anaemia and hypovolaemia of the second twin. In our case of a monochorionic twin delivery with acute TTTS the donor was born first. Early diagnosis and neonatal intervention is essential for reducing postnatal morbidity and mortality. PMID:22825763
Stein, R G; Diessner, J; Frieauff, E; Zollner, U; Rehn, M; Dietl, J; Hönig, A
Previously, both primary and secondary anti-D alloimmunizations induced by “Asian type” DEL (RHD1227A allele) were observed in two incidents. We investigated how often these alloimmunization events occur. The transfusions of any D-negative patients were investigated in the First Affiliated Hospital of Xi’an Jiaotong University Medical College, China, during the entire 2009. The antigens of D, C, c, E, and e were routinely serotyped. The “Asian type” DEL variant was genotyped and the RHD heterozygote was determined through two published methods. The changes in anti-D levels were monitored by the indirect antiglobulin test (IAT) and flow cytometry. Thirty D-negative transfused patients were included in the study. We focused on 11 recipients who were transfused with packed red blood cells (RBCs) from DEL donors at least one time. Of those 11 recipients, seven were anti-D negative before transfusion and four were anti-D positive (one patient with an autoantibody). One of the seven pre-transfusion anti-D negative patients produced a primary-response anti-D after being transfused with 400 ml of DEL blood twice. All four pre-transfusion antibody positive patients were not observed hemoglobin (Hb) levels increased, as expected after transfusions. Two patients had an increase in anti-D from 1:8 to 1:64 by IAT, which was also shown by flow cytometry. None of the patients experienced an acute hemolytic episode. Our data indicated that the primary anti-D induced by DEL transfusion or the secondary anti-D elevated by DEL in a truly D-negative patient might not be unusual. We suggest that a truly D-negative childbearing-aged woman should avoid DEL transfusion to protect her from primary anti-D allosensitization. In addition, anti-D positive recipients should also avoid DEL red cell transfusion due to the delayed hemolytic transfusion reaction (DHTR).
Shao, Chao-peng; Wang, Bao-yan; Ye, Shi-hui; Zhang, Wen-li; Xu, Hua; Zhuang, Nai-bao; Wu, Xiao-ying; Xu, Heng-gui
Priming of polymorphonuclear leukocytes (PMNs) enhances their adhesion to endothelium, the release of their granule content and their production of reactive oxygen species. These effects are etiological in transfusion related acute lung injury (TRALI) and many clinically important mediators of TRALI prime PMNs. A priming activity that develops over time in stored blood products has been shown to be due to the accumulation of lysophospatidylcholines (lyso-PCs) and has been found to be related clinically to TRALI. Lyso-PCs prime PMNs activating the G2A receptor and several inhibitors of this receptor, which could potentially be therapeutic in TRALI, have been identified. Recent work has described early steps in the signaling from the G2A receptor which has revealed potential targets for novel antagonists of lyso-PC mediated priming via G2A. Additionally, characterization of the process by which lyso-PCs are generated in stored blood products could allow development of inhibitors and additive solutions to block their formation in the first place.
Ellison, Michael A; Ambruso, Daniel R; Silliman, Christopher C
The hemolytic anemias of unknown cause can be separated into two main groups: (1) those produced by a defect in cell structure, which is usually hereditary, and (2) those due to a hemolysin of immune-body type. The hemolytic anemias associated with hypersensitivity to drugs and disease processes such as leukemia are less well understood and need further investigation. Splenectomy is the only effective treatment in congenital hemolytic jaundice and in acquired hemolytic anemia; the operation should be carried out promptly in most cases. Transfusion may be used in all varieties of hemolytic disease and is the only effective form of therapy in sickle-cell anemia and paroxysmal nocturnal hemoglobinuria.
Evans, Robert S.; Duane, Rose
The use of blood infusion in large amounts is increasing sharply. Increased knowledge of blood group antigens has alerted physicians to the possible hazards of hemolytic reactions to subgroups that must be eliminated by proper cross-matching techniques. Multiple transfusions of preserved blood often defeat their purpose in control of bleeding, for thrombocytopenia is enhanced. Careful selection of blood or preparations of plasma concentrates offer increased protection to the recipient. Plastic bag equipment increases the yield of viable platelets and keeps blood in usable condition for longer periods of storage. The use of multiple transfusions has complicated the selection of preserved blood to control pigment metabolism.
Gardner, Frank H.
Rationale Patients with chronic liver disease are at an increased risk of developing transfusion-associated acute lung injury (TRALI) from plasma containing blood products. Similarly, red blood cell transfusions have been associated with post-operative and nosocomial infections in surgical and critical care populations. Patients undergoing liver transplantation receive a large amount of cellular and plasma containing blood products, but it is presently unclear which blood components are associated with these post-operative complications. Results A retrospective cohort study of 525 consecutive liver transplant patients revealed a peri-operative TRALI incidence of 1.3% (7/525), 95%CI [0.6%–2.7%], associated with an increased hospital mortality (28.6% (2/7) vs. 2.9% (15/518), p=0.02) and intensive care unit (ICU) length of stay (2 days, [1–11] vs. 0 days [0–2], 0.03). Only high plasma containing blood products (plasma and platelets) were associated with the development of TRALI. A total of 14.3% (74/525) of patients developed a post-operative infection which was also associated with an increased in-hospital mortality (10.8% (8/74) vs. 2.0% (9/451), p < 0.01) and prolonged length of stay. Multivariate logistic regression identified the number of red blood cell units transfused (adj OR 1.08 95%CI [1.02–1.14], p<0.01), the presence of peri-operative renal dysfunction and re-operation to be significantly associated with post-operative infection. Conclusions Patients undergoing liver transplantation are at high risk of developing post-operative complications from blood transfusion. Plasma containing blood products were associated with the development of TRALI while red blood cells were associated with the development of post-operative infection in a dose dependent manner.
Benson, Alexander B.; Burton, James R.; Austin, Gregory L.; Biggins, Scott W.; Zimmerman, Michael A.; Kam, Igal; Mandell, Susan; Silliman, Christopher C.; Rosen, Hugo; Moss, Marc
Background: The goal of hemovigilance is to increase the safety and quality of blood transfusion. Identification of the adverse reactions will help in taking appropriate steps to reduce their incidence and make blood transfusion process as safe as possible. Aims: To determine the frequency and type of transfusion reactions (TRs) occurring in patients, reported to the blood bank at our institute. Materials and Methods: A retrospective review of all TRs reported to the blood bank at the All India Institute of Medical Sciences, between December 2007 and April 2012 was done. All the TRs were evaluated in the blood bank and classified using standard definitions. Results: During the study period a total of 380,658 bloods and blood components were issued by our blood bank. Out of the total 196 adverse reactions reported under the hemovigilance system, the most common type of reaction observed was allergic 55.1% (n = 108), followed by febrile non-hemolytic transfusion reaction (FNHTR) 35.7% (n = 70). Other less frequently observed reactions were Anaphylactoid reactions 5.1% (n = 10), Acute non-immune HTRs 2.6% (n = 5), Circulatory overload 0.5% (n = 1), Transfusion related acute lung injury 0.5% (n = 1), Delayed HTRs 0.5% (n = 1). Not a single case of bacterial contamination was observed. Conclusion: The frequency of TRs in our patients was found to be 0.05% (196 out of 380,658). This can be an underestimation of the true incidence because of under reporting. It should be the responsibility of the blood transfusion consultant to create awareness amongst their clinical counterpart about safe transfusion practices so that proper hemovigilance system can be achieved to provide better patient care.
Kumar, Praveen; Thapliyal, Rakesh; Coshic, Poonam; Chatterjee, Kabita
Background To avoid unnecessary blood transfusions, physiologic transfusion triggers, rather than exclusively hemoglobin-based transfusion triggers have been suggested. The objective of this study was to determine systemic and microvascular effects of using a perfluorocarbon-based oxygen carrier (PFCOC) to maintaining perfusion and oxygenation during extreme anemia. Methods The hamster (weight 55-65 g) window chamber model was used. Two isovolemic hemodilution steps were performed using 10% hydroxyethyl starch at normoxic conditions to hematocrit of 19% (5.5 gHb/dl), point where the transfusion trigger was reached. Two additional hemodilution exchanges using the PFCOC (Oxycyte™, Synthetic Blood International, Inc. Costa Mesa, CA) and increasing fraction of inspired oxygen to 1.0 were performed to reduce hematocrit to 11% (3.8 gHb/dl) and 6% (2.0 gHb/dl), respectively. No control group was used in the study, as this level of hemodilution is lethal with conventional plasma expanders. Systemic parameters, microvascular perfusion, functional capillary density and oxygen tensions across the microvascular network were measured. Results At 6% hematocrit, the PFCOC maintained mean arterial pressure, cardiac output, systemic oxygen delivery and consumption. As hematocrit was lowered from 11% to 6%, functional capillary density, calculated microvascular oxygen delivery and consumption decreased, and oxygen extraction ratio was close to 100%. Peripheral tissue oxygenation was not predicted by systemic oxygenation. Conclusions PFCOC in conjunction with hyperoxia was able to sustain organ function, and partially provide systemic oxygenation during extreme anemia over the observation period. The PFCOC can work as a bridge until red blood cells are available for transfusion, or where additional oxygen is required, notwithstanding possible limitations in peripheral tissue oxygenation.
Cabrales, Pedro; Briceno, Juan Carlos
A 16-year-old female developed a hemolytic uremic syndrome 9 months after undergoing bone marrow transplantation for acute lymphoblastic leukemia during second relapse. There appears to be no etiologic relationship between the post-transplant immune status and the hemolytic uremic syndrome. The patient succumbed later to recurrent leukemia.Copyright © 1982 S. Karger AG, Basel
W. E. Spruce; S. J. Forman; K. G. Blume; R. M. Bearman; H. Bixby; A. Ching; J. Drinkard; San Marco
A prospective, randomized double-blind study comparing the effects of irradiated and unirradiated white blood cells was conducted in 108 acute leukemia patients with life-threatening infections, refractory to antibiotics. The study demonstrated no significant improvement in 30-day survival or overall survival. Transfusion of unirradiated white cells did not compromise the patient's opportunity to undergo allogeneic stem cell transplant, nor the success rate or overall survival after allogeneic transplant. The important positive finding in this study was that the unirradiated white cells produced a significantly higher increment in circulating granulocytes and in a higher proportion of patients granulocyte count exceeded 1000 per microliter, approaching normal concentrations. The increase in the number and the improved survival of the unirradiated granulocytes suggest that this procedure might potentially be a method to improve the utility of granulocyte transfusions and merits further investigation. The study demonstrated non-inferiority for unirradiated white cells. There were no harmful effects such as graft-versus-host disease, indicating that such studies would be safe to conduct in the future. PMID:23072780
Freireich, E J; Lichtiger, B; Mattiuzzi, G; Martinez, F; Reddy, V; Kyle Wathen, J
A prospective, randomized double-blind study comparing the effects of irradiated and unirradiated white blood cells was conducted in 108 acute leukemia patients with life-threatening infections, refractory to antibiotics. The study demonstrated no significant improvement in 30-day survival or overall survival. Transfusion of unirradiated white cells did not compromise the patient's opportunity to undergo allogeneic stem cell transplant, nor the success rate or overall survival after allogeneic transplant. The important positive finding in this study was that the unirradiated white cells produced a significantly higher increment in circulating granulocytes and in a higher proportion of patients granulocyte count exceeded 1000 per microliter, approaching normal concentrations. The increase in the number and the improved survival of the unirradiated granulocytes suggest that this procedure might potentially be a method to improve the utility of granulocyte transfusions and merits further investigation. The study demonstrated non-inferiority for unirradiated white cells. There were no harmful effects such as graft-versus-host disease, indicating that such studies would be safe to conduct in the future.
Freireich, E J; Lichtiger, B; Mattiuzzi, G; Martinez, F; Reddy, V; Kyle Wathen, J
Red blood cell transfusions have reduced morbidity and mortality for patients with sickle cell disease. Transfusions can lead to erythrocyte alloimmunization, however, with serious complications for the patient including life-threatening delayed hemolytic transfusion reactions and difficulty in finding compatible units, which can cause transfusion delays. In this review, we discuss the risk factors associated with alloimmunization with emphasis on possible mechanisms that can trigger delayed hemolytic transfusion reactions in sickle cell disease, and we describe the challenges in transfusion management of these patients, including opportunities and emerging approaches for minimizing this life-threatening complication.
Ware, Russell E.
Allogeneic blood transfusion (ABT) therapy plays a major role in the case of patients with cancer. Packed red blood cells (PRBC) are given for increased oxygen-carrying capacity, platelets concentrates (PC) and fresh frozen plasma (FFP) for the cessation and prevention of bleeding due to thrombocytopenia and other defects of hemostasis associated with neoplasia. All these blood components can induce complications and/or adverse reactions in cancer patients including transfusion-associated graft versus host disease (TA-GVHD), transfusion transmitted diseases, alloimmunization to blood cell antigens, pulmonary decompensation, immunomodulation. Therefore, specific modifications such as leukocyte-reduction and irradiation of the blood components to be transfused in cancer patients should be introduced to reduce the risk of these complications. Patients undergoing hematopoietic progenitor cell (HPC) transplantation are a unique group and present complex concerns related to transfusion, including major and minor ABO incompatibility and chimeric blood cells. Therefore, transfusion for patients undergoing treatment with cellular therapies requires careful blood component selection. The process of HPC infusion itself carries many risks including DMSO toxicity and hemolytic reactions. In all areas of transfusion therapy, new advances such as pathogen inactivation and synthetic alternatives to blood components should help to increase the safety and tolerance of transfusion in cancer patients. PMID:22682136
Federici, Augusto B; Vanelli, Chiara; Arrigoni, Luisa
Evidence is presented to show that there is no hemolytic toxin producing the anemia in pernicious anemia. Partial evidence is presented to show that the periods of active blood destruction which are seen as the exception in pernicious anemia cases during a series of transfusions are due to the activity of the blood-destroying organs of the body rather than to the intrinsic weakness of the pernicious anemia blood corpuscle. It is questionable whether blood destruction is as important a factor in producing the anemia of pernicious anemia as it is at present usually assumed to be.
A case of transfusion-related acute lung injury (TRALI) that was successfully treated with extracorporeal membranous oxygenation (ECMO) is reported. A 58-year-old male patient underwent hepatectomy, and pulmonary edema occurred after the administration of fresh-frozen plasma and packed red cells. In the postoperative period, the impaired oxygenation progressively worsened, resulting in life-threatening hypoxemia, despite vigorous treatments. ECMO was therefore applied to the patient as a method of safe emergency support. Aggressive treatments under ECMO led to the successful improvement of the impaired oxygenation. TRALI is recognized as part of acute respiratory distress syndrome (ARDS). As a treatment for ARDS, ECMO does not cure the underlying disease of the lungs, however, with ECMO, TRALI, usually improves within 96 h with respiratory support. ECMO for TRALI-induced lethal hypoxemia is useful for providing time to allow the injured lung to recover. It is suggested that ECMO might be a useful option for the treatment of TRALI-induced, potentially lethal hypoxemia. PMID:19685127
Kuroda, Hiromitsu; Masuda, Yoshiki; Imaizumi, Hitoshi; Kozuka, Yuji; Asai, Yasufumi; Namiki, Akiyoshi
An open, comparative multicenter study was performed to evaluate the efficacy and safety of azithromycin (10 mg/kg) given once daily for three days in comparison with cefaclor (30 mg/kg) divided into three daily doses and given for a period of ten days. One hundred and twenty-two children aged 1-12 years with clinical symptoms of group A beta-hemolytic streptococcal tonsillopharyngitis and a positive throat culture were randomly allocated to the treatment groups. Overall, the clinical success (cure or improvement) of both regimens was identical in the evaluable patients (86.3%, 44 of 51 patients in either treatment group). In contrast, bacterial eradication after completion of treatment was lower with azithromycin than with cefaclor. Possible reasons for this discrepancy between clinical success and eradication rates could be antibiotic resistance, pre-disease carriage or insufficient dosage. Both agents were well tolerated; only mild or moderate side effects most frequently involving the gastrointestinal tract, were recorded in either therapy group. PMID:9707305
Cremer, J; Wallrauch, C; Milatovic, D; Braveny, I
. Serum erythropoietin (EPO) levels were measured in ten previously non-transfused children with hemolytic uremic syndrome\\u000a (HUS). Complete blood cell count, serum EPO, and renal function tests were carried out upon admission and weekly thereafter.\\u000a Blood samples were obtained: (1) prior to the first transfusion; (2) after the first transfusion but before recovery from\\u000a renal failure; (3) during the recovery
Ramon Exeni; Hugo Donato; Pablo Rendo; María Antonuccio; María Cristina Rapetti; Irene Grimoldi; Andrea Exeni; Ana de Galvagni; Emilia Trepacka; Alicia Amore
Background In aortic surgery bleeding complications can be fatal. Therefore, rotational thromboelastometry(ROTEM™)-based coagulation management was introduced. Methods After 5 cases of acute type A aortic dissection and aortic arch replacement had been treated based on ROTEM findings (ROTEM group; RG), 5 cases without ROTEM were matched as control group (CG). CG treatment was based on conventional tests and clinical findings. Blood component and coagulation factor requirements, ventilation time, duration of stay at intensive care unit (ICU), hospitalization, and thrombotic or bleeding incidents as well as transfusion-associated costs were compared. Results Administration of blood products and coagulation factor concentrates, ventilation time, ICU length of stay, and hospitalization tended to be lower in RG. Postoperative plasma transfusion (p = 0.038), recognized incidents (p = 0.048), and resulting costs on coagulation treatment (p = 0.049) were significantly reduced. Conclusion Our data suggest that ROTEM-based coagulation management can reduce transfusion requirements and corresponding costs in patients with aortic arch replacement. These data has to be confirmed by prospective randomized trials.
Hanke, Alexander A.; Herold, Ulf; Dirkmann, Daniel; Tsagakis, Konstantinos; Jakob, Heinz; Gorlinger, Klaus
Transfusion-related acute lung injury (TRALI), a form of noncardiogenic pulmonary edema that develops during or within 6 h after a blood transfusion, is the most frequent cause of transfusion-associated death in the United States. Because development of TRALI is associated with donor antibodies (Abs) reactive with recipient major histocompatibility complex (MHC), a mouse model has been studied in which TRALI-like disease is caused by injecting mice with anti–MHC class I monoclonal Ab (mAb). Previous publications with this model have concluded that disease is caused by FcR-dependent activation of neutrophils and platelets, with production of reactive oxygen species that damage pulmonary vascular endothelium. In this study, we confirm the role of reactive oxygen species in the pathogenesis of this mouse model of TRALI and show ultrastructural evidence of pulmonary vascular injury within 5 min of anti–MHC class I mAb injection. However, we demonstrate that disease induction in this model involves macrophages rather than neutrophils or platelets, activation of complement and production of C5a rather than activation of Fc?RI, Fc?RIII, or Fc?RIV, and binding of anti–MHC class I mAb to non-BM–derived cells such as pulmonary vascular endothelium. These observations have important implications for the prevention and treatment of TRALI.
Strait, Richard T.; Hicks, Wyenona; Barasa, Nathaniel; Mahler, Ashley; Khodoun, Marat; Kohl, Jorg; Stringer, Keith; Witte, David; Van Rooijen, Nico; Susskind, Brian M.
Shiga toxin (Stx)-producing Escherichia coli (STEC) infection is associated with a broad spectrum of clinical manifestations that include diarrhea, hemorrhagic colitis, and hemolytic uremic syndrome (HUS). Systemic Stx toxemia is considered to be central to the genesis of HUS. Distinct methods have been used to evaluate anti-Stx response for immunodiagnostic or epidemiological analysis of HUS cases. The development of enzyme-linked immunosorbent assay (ELISA) and western blot (WB) assay to detect the presence of specific antibodies to Stx has introduced important advantages for serodiagnosis of HUS. However, application of these methods for seroepidemiological studies in Argentina has been limited. The aim of this work was to develop an ELISA to detect antibodies against the B subunit of Stx2, and a WB to evaluate antibodies against both subunits of Stx2 and Stx1, in order to analyze the pertinence and effectiveness of these techniques in the Argentinean population. We studied 72 normal healthy children (NHC) and 105 HUS patients of the urban pediatric population from the surrounding area of Buenos Aires city. Using the WB method we detected 67% of plasma from NHC reactive for Stx2, but only 8% for Stx1. These results are in agreement with the broad circulation of Stx2-expressing STEC in Argentina and the endemic behavior of HUS in this country. Moreover, the simultaneous evaluation by the two methods allowed us to differentiate acute HUS patients from NHC with a great specificity and accuracy, in order to confirm the HUS etiology when pathogenic bacteria were not isolated from stools.
Fernandez-Brando, Romina J.; Bentancor, Leticia V.; Mejias, Maria Pilar; Ramos, Maria Victoria; Exeni, Andrea; Exeni, Claudia; del Carmen Laso, Maria; Exeni, Ramon; Isturiz, Martin A.; Palermo, Marina S.
The Accuracy of Natriuretic Peptides (BNP and NT-pro-BNP) in the Differentiation between Transfusion Related Acute Lung Injury (TRALI) and Transfusion Related Circulatory Overload (TACO) in the Critically Ill
BACKGROUND The diagnostic workup of TRALI requires an exclusion of TACO. Brain natriuretic peptide (BNP) and N-terminal pro-brain natriuretic (NT-pro-BNP) accurately diagnosed TACO in preliminary studies that did not include patients with TRALI. STUDY DESIGN AND METHODS In this prospective cohort study two critical care experts blinded to serum levels of BNP and NT-pro-BNP determined the diagnosis of TRALI, TACO, and possible TRALI based on the consensus conference definitions. The accuracy of BNP and NT-pro-BNP was assessed based on the area under the receiver operating curve (AUC). RESULTS Of 115 patients who developed acute pulmonary edema after transfusion, 34 were identified with TRALI, 31 with possible TRALI, and 50 with TACO. Median BNP was 375pg/mL (interquartile range [IQR], 122.5 to 780.5pg/mL) in TRALI, 446pg/mL (IQR, 128 to 743.3pg/mL) in possible TRALI and 559pg/mL (IQR, 287.8 to 1347.8pg/mL) in TACO patients (p=0.038). The NT-pro-BNP levels among patients with TRALI, possible TRALI and TACO differed significantly with a median value of 1558.5pg/mL(IQR, 628.5 to 5114pg/mL), 2349pg/mL(IQR, 919 to 4610pg/mL) and 5197pg/mL(IQR, 1695 to 15714pg/mL)(p=0.0036), respectively. The accuracy of BNP and NT-pro-BNP to diagnose TACO was moderate with an area under curve (AUC) of 0.63(95% confidence interval [CI] 0.51 to 0.74) and 0.70(95%CI 0.59 to 0.80). CONCLUSIONS Natriuretic peptides are of limited diagnostic value in a differential diagnosis of pulmonary edema after transfusion in the critically ill patients.
Li, Guangxi; Daniels, Craig E.; Kojicic, Marija; Krpata, Tami; Wilson, Greg A; Winters, Jeffrey L; Moore, S Breanndan; Gajic, Ognjen
A 4-year-old boy presented with enteroviral infection complicated with atypical hemolytic uremic syndrome (aHUS). Enterovirus RNA was detected by reverse transcription polymerase chain reaction (RT-PCR) of both blood and kidney biopsy specimens. A survey of the complement system did not reveal a specific complement defect. Supportive therapy with blood components transfusion, plasma therapy, and immunosuppressants was administered, however, renal function did not recover. The results of this report demonstrate that the enterovirus is the cause of aHUS. PMID:23597514
Lee, Ming-Dar; Tzen, Chin-Yuan; Lin, Chun-Chen; Huang, Fu-Yuan; Liu, Hsi-Che; Tsai, Jeng-Daw
Although blood transfusion is an extremely important therapeutic procedure that usually proceeds without complications, there are some risks associated with donated blood. Investigations into the causes of transfusion reactions and their prevention are important issues for transfusion therapy. In addition to nucleic acid amplification testing (NAT) for infectious diseases and the irradiation of blood to prevent post-transfusion GVHD, prestorage leukocyte reduction and diversion of the first part of the donation of blood were recently introduced into transfusion therapy. This symposium, entitled "Immunoreaction and blood transfusion", reviewed the immune responses associated with blood transfusion, which is probably the most frequent medical procedure performed in allogeneic organ transplantation, with four themes provided by the four featured invited speakers: transfusion-related acute lung injury (TRALI) and transfusion-associated circulatory overload (TACO), high-dose intravenous immunoglobulin therapy for chronic inflammatory demyelinating polyradiculoneuropathy, transfusion-transmitted infectious disease surveillance, and transfusion-related immunomodulation. PMID:23947177
Kawabe, Tsutomu; Matsushita, Tadashi
Escherichia coli strains producing Shiga toxins (Stxs) colonize the lower gastrointestinal tract and cause watery diarrhea, hemorrhagic colitis,\\u000a and hemolytic-uremic syndrome (HUS). HUS is characterized by hemolytic anemia, thrombocytopenia, and acute renal failure.\\u000a Oliguria associated with acute tubular necrosis and microangiopathic thrombosis has been reported as the most common cause\\u000a of renal failure in Argentinean children. Our study was undertaken
Elsa Zotta; Nestor Lago; Federico Ochoa; Horacio A. Repetto; Cristina Ibarra
Transfusion-related acute lung injury (TRALI) induced by donor-derived anti-HLA antibodies in aplastic anemia: possible priming effect of granulocyte-colony stimulating factor (G-CSF) on the recipient neutrophils.
Transfusion-related acute lung injury (TRALI) is currently the leading cause of transfusion-related death. A 67-year-old man with severe aplastic anemia developed TRALI, consisting of acute respiratory insufficiency with severe hypoxia and diffuse pulmonary infiltration 2 hours after the transfusion of platelet concentrates. Although he required intensive respiratory support, he promptly recovered within 4 days. The presence of anti-HLA antibody (anti-HLA B52) in the donated blood product was demonstrated, and a lymphocytotoxicity test disclosed antibody-mediated cytotoxicity against the patient's cells. Furthermore, administration of granulocyte-colony stimulating factor was suggested to predispose the patient to TRALI by priming the neutrophils. PMID:19915300
Hishizawa, Masakatsu; Mitsuhashi, Ryuichi; Ohno, Tatsuharu
INTRODUCTION: Autoimmune hemolytic anemia in children younger than 2 years of age is usually characterized by a severe course, with a mortality rate of approximately 10%. The prolonged immunosuppression following specific treatment may be associated with a high risk of developing severe infections. Recently, the use of monoclonal antibodies (rituximab) has allowed sustained remissions to be obtained in the majority
Chiara Beretta; Veronica Leoni; Mario Renato Rossi; Momcilo Jankovic; Nicolo Patroniti; Giuseppe Foti; Ettore Biagi
The erythrocyte lifespan in haemolytic anemia is shortened while erythropoesis is increased. Important labaratory findings are increased reticulocytes, LDH, indirect bilirubin and a decreased haptoglobin level. The most important diagnostic tool for further work up of hemolytic anemia is the direct antiglobulin test (DAT, Coombs test) to differentiate autoimmune hemolytic anemia (AIHA) from other causes. Another important group are fragmentation syndroms (hemolytic uremic syndrome and thrombotic thrombocytopenic purpura). In these forms of haemolytic anemia fragmented red blood cells can be found in the blood smear together with thrombocytopenia. A severe problem in paroxysmal nocturnal hematuria is the incidence of thrombosis. The following review describes the most important forms of hemolytic anemia in the adult and the diagnostic and therapeutic strategies. PMID:22048936
Müller, A; Zimmermann, R; Krause, S W
Platelets are activated in sickle cell disease (SCD), and particularly during vaso-occlusive episodes (VOE). Thrombospondin-1 (TSP1), a major secretory product of activated platelets, is increased in the circulation in VOE and binds to sickle red blood cells (RBC) promoting vascular adhesion. Thus, we hypothesized that TSP1 may represent a plasma biomarker of disease severity in SCD. We tested the plasma collected from patients in steady state (n = 27) and VOE (n = 14), as well as healthy controls (n = 17) at the University of Pittsburgh Medical Center (UPMC), and from patients in steady state enrolled in the walk-PHaSST clinical trial (n = 483). We found that TSP1 levels were increased in VOE in the UPMC cohort. Among steady-state patients at UPMC, TSP1 values correlated positively with lifetime history of acute chest syndrome (r = 0.72, P < 0.0001) and hemoglobin concentration (r = 0.49, P = 0.01), and negatively with markers of hemolysis, such as LDH (r = ?0.50, P = 0.009). Analysis of the walk-PHaSST cohort also showed a positive association between TSP1 levels and hydroxyurea use (r = 0.14, P = 0.003), and confirmed the negative associations with the severity of hemolysis. Our results suggest that TSP1 levels are associated with more VOE, hydroxyurea use and lower rates of hemolysis. High TSP1 concentrations may indicate higher risk of the viscosity/vaso-occlusion phenotype of SCD.
Novelli, Enrico M; Kato, Gregory J.; Ragni, Margaret V.; Zhang, Yingze; Hildesheim, Mariana E.; Nouraie, Mehdi; Barge, Suchitra; Meyer, Michael P.; Hassett, Andrea Cortese; Gordeuk, Victor R.; Gladwin, Mark T.; Isenberg, Jeffrey S.
Platelets are activated in sickle cell disease (SCD), and particularly during vaso-occlusive episodes (VOE). Thrombospondin-1 (TSP1), a major secretory product of activated platelets, is increased in the circulation in VOE and binds to sickle red blood cells (RBC) promoting vascular adhesion. Thus, we hypothesized that TSP1 may represent a plasma biomarker of disease severity in SCD. We tested the plasma collected from patients in steady state (n = 27) and VOE (n = 14), as well as healthy controls (n = 17) at the University of Pittsburgh Medical Center (UPMC), and from patients in steady state enrolled in the walk-PHaSST clinical trial (n = 483). We found that TSP1 levels were increased in VOE in the UPMC cohort. Among steady-state patients at UPMC, TSP1 values correlated positively with lifetime history of acute chest syndrome (r = 0.72, P < 0.0001) and hemoglobin concentration (r = 0.49, P = 0.01), and negatively with markers of hemolysis, such as LDH (r = -0.50, P = 0.009). Analysis of the walk-PHaSST cohort also showed a positive association between TSP1 levels and hydroxyurea use (r = 0.14, P = 0.003), and confirmed the negative associations with the severity of hemolysis. Our results suggest that TSP1 levels are associated with more VOE, hydroxyurea use and lower rates of hemolysis. High TSP1 concentrations may indicate higher risk of the viscosity/vaso-occlusion phenotype of SCD. PMID:22318901
Novelli, Enrico M; Kato, Gregory J; Ragni, Margaret V; Zhang, Yingze; Hildesheim, Mariana E; Nouraie, Mehdi; Barge, Suchitra; Meyer, Michael P; Hassett, Andrea Cortese; Gordeuk, Victor R; Gladwin, Mark T; Isenberg, Jeffrey S
Thirty-two first renal transplantations with cadaveric allografts were reviewed to see how many of the recipients had received blood transfusions preoperatively. There was a significant difference in transplant survival between patients who had and patients who had not received blood transfusion before transplantation; this difference was entirely due to acute rejection within three months after transplantation in patients who had
R W Blamey; M S Knapp; R P Burden; M Salisbury
These proceedings contain 24 selections, including papers presented at the conference of American Red Cross held in May 1985, on the Subject of transfusion medicine. Some of the titles are: Fluosol\\/sup R\\/-DA in Radiation Therapy; Expression of Cloned Human Factor VIII and the Molecular Basis of Gene Defects that Cause Hemophilia; DNA-Probing Assay in the Detection of Hepatitis B Virus
K. Murawski; F. Peetoom
These proceedings contain 24 selections, including papers presented at the conference of American Red Cross held in May 1985, on the Subject of transfusion medicine. Some of the titles are: Fluosol/sup R/-DA in Radiation Therapy; Expression of Cloned Human Factor VIII and the Molecular Basis of Gene Defects that Cause Hemophilia; DNA-Probing Assay in the Detection of Hepatitis B Virus Genome in Human Peripheral Blood Cells; and Monoclonal Antibodies: Convergence of Technology and Application.
Murawski, K.; Peetoom, F.
The hemolytic uremic syndrome (HUS) is the most-common cause of acute renal failure in children. Several researchers have reported the presence of neutrophil (PMN) activating cytokines, such as interleukin-8 and tumor necrosis factor-alpha, in the sera of HUS patients. Moreover, PMN-derived products, such as elastase, were increased. These observations have lead to the hypothesis that activated PMN could act as mediators of endothelial damage. The objective of this investigation was to directly evaluate the activation status of peripheral PMN from children with HUS. For this purpose, 12 children with typical HUS were bled during the acute period, before dialysis and/or transfusion, and 8 of them were also bled after 1 month follow-up. Additionally, blood samples from healthy control children admitted for routine surgical procedures, chronic uremic children, and neutrophilic children with acute infections not related to HUS were collected and processed in an identical manner. The function and membrane activation markers of PMN from these groups were evaluated. We found that during the acute period of HUS, PMN had reduced expression of FcgammaRIII (CD16) and CD11b, were degranulated, and exhibited an impaired antibody-dependent cellular cytotoxicity. These parameters returned to normal after clinical recuperation. We conclude that PMN activation in HUS patients is a very early and transient event, and upon hospitalization before dialysis PMN show a phenotype and functional pattern of partial deactivation. PMID:12042890
Fernández, Gabriela C; Rubel, Carolina; Barrionuevo, Paula; López, Laura; Ramirez, Flavia; Díaz, Mario; Isturiz, Martín A; Palermo, Marina S
A cell-bound hemolytic activity was found in several strains of Serratia marcescens. One Serratia cell per ten erythrocytes was sufficient to cause complete lysis of human erythrocytes within 2 h in the liquid assay. The hemolytic activity resided in the membrane fraction and could be inactivated by incubating cells with proteases. The hemolytic activity was greatly enhanced in actively metabolizing
Volkmar Braun; Hannelore Günther; Burkard Neuß; Christiane Tautz
Decreased fetal movement (DFM) perceived by pregnant women sometimes indicates imminent fetal jeopardy. It is unknown whether this also holds true for twin pregnancy. A 27-year-old primiparous woman with monochorionic diamniotic (MD) pregnancy had a slight difference of amniotic fluid volume at 31(2/7) weeks of gestation. DFM only in one twin at 31(4/7) weeks of gestation prompted her to receive urgent consultation. Since cardiotocogram indicated absent variability of one twin, we performed Cesarean section. Male infants weighing 2060?g and 1578?g were delivered; hemoglobin was 20.7 versus 10.8?g/dL, respectively; cardiothoracic ratio was 70% versus 44%, respectively, indicating acute twin-to-twin transfusion syndrome (TTTS). The recipient infant had heart failure, which was still observed at 1 month postpartum. In conclusion, maternal perception of DFM indicated imminent fetal death or jeopardy caused by acute TTTS, suggesting that education regarding DFM for women with twin pregnancy may be clinically important. PMID:23984131
Suzuki, Hirotada; Kuwata, Tomoyuki; Ohkuchi, Akihide; Yada, Yukari; Matsubara, Shigeki; Suzuki, Mitsuaki
We previously reported that ultraviolet light B (UVB)-treated human platelets (hPLTs) can cause acute lung injury (ALI) in a two-event SCID mouse model in which the predisposing event was Lipopolysaccharide (LPS) injection and the second event was infusion of UVB-treated hPLTs. To delineate contributions of host mouse platelets (mPLTs) and neutrophils in the pathogenesis of ALI in this mouse model, we depleted mPLTs or neutrophils and measured hPLT accumulation in the lung. We also assessed lung injury by protein content in bronchoalveolar lavage fluid (BALF). LPS injection followed by infusion of UVB-treated hPLTs resulted in sequestration of both mPLTs and hPLTs in the lungs of SCID mice, although the numbers of neutrophils in the lung were not significantly different from the control group. Depletion of mouse neutrophils caused only a mild reduction in UVB-hPLTs accumulation in the lungs and a mild reduction in protein content in BALF. In comparison, depletion of mPLTs almost completely abolished hPLTs accumulation in the lung and significantly reduced protein content in BALF. UVB-treated hPLTs bound to host mPLTs, but did not bind to neutrophils in the lung. Aspirin treatment of hPLTs in vitro abolished hPLT accumulation in the lung and protected mice from lung injury. Our data indicate that host mPLTs accumulated in the lungs in response to an inflammatory challenge and subsequently mediated the attachment of transfused UVB-hPLTs. Neutrophils also recruited a small percentage of platelets to the lung. These findings may help develop therapeutic strategies for ALI which could potentially result from transfusion of UV illuminated platelets.
Chi, Xuan; Zhi, Li; Gelderman, Monique P.; Vostal, Jaroslav G.
Hemolytic uremic syndrome (HUS) is a rare disease characterized by microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. It is usually secondary to infections by strains of Escherichia coli (STEC) that produce Shiga-like toxin. In about 10% of patients, no STEC infections are reported. In these cases of atypical HUS (aHUS), mutations in genes encoding proteins of the complement system have been described. Atypical HUS is characterized by poor prognosis and by high risk of posttransplant recurrence which greatly depends on the specific gene mutation involved in the disease. Plasma therapy, eculizumab treatment and, in some cases, combined liver-kidney transplant have been used to prevent and/or treat posttransplant aHUS recurrences. PMID:22760880
Valoti, Elisabetta; Alberti, Marta; Noris, Marina
Background: Fatigue in anaemia is empirically reduced by blood transfusion. Long storage time of red cells may be associated with immunomodulatory effects, and blood stored for a long time may cause tissue hypoxia upon transfusion. Patients and Methods: 22 patients admitted with haemoglobin < 6.0 mmol\\/l, complaints of fatigue and no active bleeding were included. Eleven patients received two red
Tommie Mynster; Morten Hanefeld Dziegiel; Kristian Kofoed
The Serious Hazards of Transfusion (SHOT) UK confidential haemovigilance reporting scheme began in 1996. Over the 16 years of reporting, the evidence gathered has prompted changes in transfusion practice from the selection and management of donors to changes in hospital practice, particularly better education and training. However, half or more reports relate to errors in the transfusion process despite the introduction of several measures to improve practice. Transfusion in the UK is very safe: 2·9 million components were issued in 2012, and very few deaths are related to transfusion. The risk of death from transfusion as estimated from SHOT data in 2012 is 1 in 322 580 components issued and for major morbidity, 1 in 21 413 components issued; the risk of transfusion-transmitted infection is much lower. Acute transfusion reactions and transfusion-associated circulatory overload carry the highest risk for morbidity and death. The high rate of participation in SHOT by National Health Service organizations, 99·5%, is encouraging. Despite the very useful information gained about transfusion reactions, the main risks remain human factors. The recommendations on reduction of errors through a 'back to basics' approach from the first annual SHOT report remain absolutely relevant today. PMID:24032719
Bolton-Maggs, Paula H B; Cohen, Hannah
Trauma is the leading cause of death in young adults and acute blood loss contributes to a large portion of mortality in the early post-trauma period. The recognition of lethal triad of coagulopathy, hypothermia and acidosis has led to the concepts of damage control surgery and resuscitation. Recent experience with managing polytrauma victims from the Iraq and Afghanistan wars has led to a few significant changes in clinical practice. Simultaneously, transfusion practices in the civilian settings have also been extensively studied retrospectively and prospectively in the last decade. Early treatment of coagulopathy with a high ratio of fresh frozen plasma and platelets to packed red blood cells (FFP:platelet:RBC), prevention and early correction of hypothermia and acidosis, monitoring of hemostasis using point of care tests like thromoboelastometry, use of recombinant activated factor VII, antifibrinolytic drugs like tranexamic acid are just some of the emerging trends. Further studies, especially in the civilian trauma centers, are needed to confirm the lessons learned in the military environment. Identification of patients likely to need massive transfusion followed by immediate preventive and therapeutic interventions to prevent the development of coagulopathy could help in reducing the morbidity and mortality associated with uncontrolled hemorrhage in trauma patients.
Bhananker, Sanjay M; Ramaiah, Ramesh
The aim of this study was to assess the level of hepcidin in hereditary chronic hemolytic anemias and to correlate the serum hepcidin levels to the need for blood transfusions (frequency of blood transfusions and the serum ferritin level). Seventy pediatric patients with hereditary chronic hemolytic anemias, attending to hematology clinics of Cairo University and Misr University for Science and Technology (MUST) hospitals were the subjects of this study [53 patients with ?-thalassemia major (?-TM), 10 patients with ?-thalassemia intermedia (?-TI), four patients with congenital spherocytosis and three patients with sickle cell disease) (38 males and 32 females)]; their ages ranged from 1-14 years. Seventy normal children, age- and sex-matched, served as the control group. The results of this study revealed decreased hepcidin levels in patients (all types of congenital chronic hemolytic anemias) [mean ± SD (standard deviation) = 22.9 ± 6.0] compared to controls (mean ± SD = 132.4 ± 16.7) with highly significant statistical difference in between. Hepcidin levels were higher in ?-TM patients (mean ± SD = 23.7 ± 6.2) than in ?-TI patients (mean ± SD = 21.8 ± 4.0), the hepcidin to ferritin ratio was significantly less than one. In ?-TM patients, the mean ± SD was 0.03 ± 0.004, and in ?-TI patients the mean ± SD = 0.025 ± 0.002, with highly significant statistical difference with hepcidin-to-ferritin ratios in controls being mean ± SD = 2.3 ± 0.7. Hepcidin and hepcidin/ferritin ratios can be used as good markers of hemolytic anemia and iron overload as they have very high sensitivity (99.0 and 99.0%, respectively) and very high specificity (98.0 and 97.0%, respectively). Our findings highlight the potential usefulness of hepcidin measurement as a diagnostic tool. The use of hepcidin as an adjuvant therapy with iron chelators is important as it has a vital role in combating hemosidrosis. PMID:23088733
El Beshlawy, Amal; Alaraby, Ibrahim; Abdel Kader, Mohamed S E M; Ahmed, Dina H; Abdelrahman, Hossam E M
Effects of large dosages of amoxicillin/clavulanate or azithromycin on nasopharyngeal carriage of Streptococcus pneumoniae, Haemophilus influenzae, nonpneumococcal alpha-hemolytic streptococci, and Staphylococcus aureus in children with acute otitis media.
Prior use of antibiotics is associated with carriage of resistant bacteria. Colonization by Streptococcus pneumoniae, Haemophilus influenzae, nonpneumococcal alpha-hemolytic streptococci (NPAHS), and Staphylococcus aureus was evaluated in children receiving antibiotic therapy for acute otitis media and in untreated, healthy control subjects. Children were randomly assigned to receive either amoxicillin/clavulanate (90 mg/kg per day) or azithromycin. Swabs were obtained before initiating therapy and again 2 weeks and 2 months after initiating therapy. We also obtained swabs from control subjects at the time of enrollment and 2 weeks and 2 months after enrollment. The decrease in the rate of carriage of S. pneumoniae and H. influenzae at 2 weeks was significant only in the amoxicillin/clavulanate group (P<.001 and P=.005, respectively). The rate of nasopharyngeal colonization with NPAHS among treated patients increased from 23% to 39% at 2 months (P=.01). This increase was similar for both treatment groups. These results suggest that the competitive balance between organisms is altered by antibiotic therapy. PMID:11981724
Ghaffar, Faryal; Muniz, Luz Stella; Katz, Kathy; Smith, Jeanette L; Shouse, Theresa; Davis, Phyllis; McCracken, George H
Transfusion of Phosphoenolpyruvate-treated Blood Increases Oxygen Consumption in Acute Hemorrhage 1 1 The opinions or assertions contained herein are the private views of the authors and are not to be construed as reflecting the views of the Department of the Air Force or the Department of Defense
Background: Incubating blood with phosphoenolpyruvate decreases hemoglobin oxygen affinity (HOA). This study compared transfusion with phosphoenolpyruvate-treated blood and conventionally stored blood on oxygen consumption in acutely anemic dogs.Methods: Dogs underwent isovolemic hemodilution (hematocrit = 10%). After 1 hour they were transfused to a hematocrit of 18% with control or phosphoenolpyruvate treated blood. Cardiac output, co-oxymetry, and hemoglobin P50 measurements allowed
Rhonda L Cornum; R. Russell Martin; William C Bandy
Gene analysis in Japanese patients with congenital hemolytic anemia due to red cell membrane disorders, thalassemias, unstable hemoglobinopathies and red cell enzymopathies were summarized. In hereditary spherocytosis, twenty-four mutations of band 3, five mutations of protein 4.2 and twenty mutations of ankyrin have been identified. In beta thalassemia, fourty-seven mutations of beta globin have been found, and ten mutations among them comprise 80% of beta thalassemia patients in Japan. Most common alpha0 and alpha+ thalassemia are--SEA and--alpha3.7, respectively. Fourty glucose-6-phosphate dehydrogenase mutations and twenty-three pyruvate kinase mutations have been identified, allowing a better understanding of the structure-function relationships of these enzymes. PMID:15773339
We describe a case of pulmonary bleeding and subsequent acute respiratory distress syndrome (ARDS) in a 20-month-old female suffering from a typical postdiarrheal hemolytic-uremic syndrome (HUS). Acute renal failure was treated early by peritoneal dialysis. It is of interest to underline that thrombocytopenia or any coagulative impairment was absent when this complication occurred, and spontaneous diuresis recovery was ongoing. All
M. Piastra; A. Ruggiero; A. Langer; E. Caresta; A. Chiaretti; S. Pulitanò; G. Polidori; R. Riccardi
DEAP-HUS [Deficiency of CFHR (complement factor H-related) plasma proteins and Autoantibody Positive form of Hemolytic Uremic Syndrome] represents a novel subtype of hemolytic uremic syndrome (HUS) with unique characteristics. It affects children and\\u000a requires special clinical attention in terms of diagnosis and therapy. DEAP-HUS and other atypical forms of HUS share common\\u000a features, such as microangiopathic hemolytic anemia, acute renal
Peter F. Zipfel; Christoph Mache; Dominik Müller; Christoph Licht; Marianne Wigger; Christine Skerka
The classical triad of hemolytic uremic syndrome (microangiopathic hemolytic anemia, severe thrombopenia, and renal failure) developed de novo in three of our renal transplanted patients under cyclosporin A treatment. The predominant morphologic findings in the grafts consisted of glomerular and arteriolar thrombosis as well as arteriolonecrosis, all features of the syndrome. In one instance, ischemic bowel disease supervened after graft removal and was associated with persistent low grade microangiopathic process. De novo hemolytic uremic syndrome has been reported in patients treated with cyclosporin A following bone marrow or liver transplantation as well as in a few renal graft recipients. This peculiar form of cyclosporin A nephrotoxicity should not be confused with acute rejection of the renal transplant. PMID:3304591
Giroux, L; Smeesters, C; Corman, J; Paquin, F; Allaire, G; St-Louis, G; Daloze, P
Hemolytic uremic syndrome (HUS) is defined by the triad of mechanical hemolytic anemia, thrombocytopenia and renal impairment. Atypical HUS (aHUS) defines non Shiga-toxin-HUS and even if some authors include secondary aHUS due to Streptococcus pneumoniae or other causes, aHUS designates a primary disease due to a disorder in complement alternative pathway regulation. Atypical HUS represents 5 -10% of HUS in children, but the majority of HUS in adults. The incidence of complement-aHUS is not known precisely. However, more than 1000 aHUS patients investigated for complement abnormalities have been reported. Onset is from the neonatal period to the adult age. Most patients present with hemolytic anemia, thrombocytopenia and renal failure and 20% have extra renal manifestations. Two to 10% die and one third progress to end-stage renal failure at first episode. Half of patients have relapses. Mutations in the genes encoding complement regulatory proteins factor H, membrane cofactor protein (MCP), factor I or thrombomodulin have been demonstrated in 20-30%, 5-15%, 4-10% and 3-5% of patients respectively, and mutations in the genes of C3 convertase proteins, C3 and factor B, in 2-10% and 1-4%. In addition, 6-10% of patients have anti-factor H antibodies. Diagnosis of aHUS relies on 1) No associated disease 2) No criteria for Shigatoxin-HUS (stool culture and PCR for Shiga-toxins; serology for anti-lipopolysaccharides antibodies) 3) No criteria for thrombotic thrombocytopenic purpura (serum ADAMTS 13 activity > 10%). Investigation of the complement system is required (C3, C4, factor H and factor I plasma concentration, MCP expression on leukocytes and anti-factor H antibodies; genetic screening to identify risk factors). The disease is familial in approximately 20% of pedigrees, with an autosomal recessive or dominant mode of transmission. As penetrance of the disease is 50%, genetic counseling is difficult. Plasmatherapy has been first line treatment until presently, without unquestionable demonstration of efficiency. There is a high risk of post-transplant recurrence, except in MCP-HUS. Case reports and two phase II trials show an impressive efficacy of the complement C5 blocker eculizumab, suggesting it will be the next standard of care. Except for patients treated by intensive plasmatherapy or eculizumab, the worst prognosis is in factor H-HUS, as mortality can reach 20% and 50% of survivors do not recover renal function. Half of factor I-HUS progress to end-stage renal failure. Conversely, most patients with MCP-HUS have preserved renal function. Anti-factor H antibodies-HUS has favourable outcome if treated early.
A model to examine the in vivo relationship of acute phase serum amyloid A (SAA) to spleen cholesterol mobilisation was devised. Reticuloendothelial cells in vivo were loaded with a known quantity of cholesterol (1.5 mg) by infusing fragmented red blood cell membranes, which consist of approximately 50% cholesterol by dry weight. Following infusion, 7% of the infused cholesterol was in the spleen and significantly increased (by 35%) spleen cholesterol concentration above the baseline. An acute inflammatory reaction was induced by the subcutaneous injection of AgNO(3) which also raised spleen cholesterol values, but not significantly. Both treatments were also administered together and the increase in spleen cholesterol concentration after 1 h was equivalent to the sum of the individual treatments. In all the treatment groups, the spleen cholesterol concentration and the plasma SAA values were then followed over a period of 24 h. In all treatment groups the spleen cholesterol values fell to baseline values primarily between 18 and 24 h which coincided with significantly raised levels of plasma SAA. In the case of the dual treatment, between 4 and 18 h, SAA increased from 92.1 +/- 12.3 to 478 +/- 58.3 microg/ml, respectively and depletion of spleen cholesterol occurred gradually reaching baseline values after 24 h. The significant flux of cholesterol though the spleen raises the distinct possibility that the spleen is much more involved in cholesterol metabolism than previously appreciated. Furthermore, the speed with which plasma SAA increases following the infusion of fragmented red blood cell membranes and the role that SAA plays in cholesterol mobilisation raise issues that may be relevant to alterations in plasma acute phase protein and lipid parameters in patients undergoing transfusions or suffering from hemolytic disorders. PMID:19065296
Li, Chunyan; Kisilevsky, Robert
Transfusion of red blood cells can be a life-saving therapy both for patients with chronic anemias and for those who are critically ill with acute blood loss. However, transfusion has been associated with significant morbidity. Chronic transfusion results in accumulation of excess iron that surpasses the binding capacity of the major iron transport protein, transferrin. The resulting non–transferrin bound iron
Caroline P. Ozment; Jennifer L. Turi
The influence of myocardial infarction (MI) on the transfusion requirements of transfusion dependent patients has not been previously studied. We studied thirty frequently transfused patients on long-term hemodialysis and a similar number of age and sex m...
J. P. Crowley C. R. Valeri J. Chazan
Blood and blood components are considered drugs because they are used in the treatment of diseases. As with any drug, adverse effects may occur, necessitating careful consideration of therapy. Like any other therapeutic decision, the need for transfusion should be considered on the basis of risks and benefits and alternative treatments available to avoid over- and under-transfusion. This review is focused on the blood transfusion protocol in trauma patients with hemorrhagic shock. Besides, issues related to emergency and massive transfusion have also been elaborated. We conducted a comprehensive MEDLINE search and reviewed the relevant literature, with particular reference to emergency medical care in trauma.
Kaur, Paramjit; Basu, Sabita; Kaur, Gagandeep; Kaur, Ravneet
Marked variability in transfusion practice exists in cardiac surgical patients, with consumption of approximately 20% of the worldwide allogeneic blood supply. Observational studies have reported an association between red blood cell transfusion and adverse outcome, including pulmonary complications, in cardiac surgery. Tuinman and colleagues report that transfusions were associated with activation of pulmonary inflammation and coagulation by measurement of biomarkers in bronchoalveolar lavage fluid, and suggest that transfusion may be a mediator of acute lung injury. This study provides interesting preliminary data, but is limited by multiple confounding variables (plasma transfusion, use of anticoagulants and heparin antagonists) and the small sample size. A large multicenter, prospective, randomized clinical trial regarding the safety (inclusive of pulmonary complications) and efficacy of red blood cell transfusion in cardiac surgery is needed.
Anaphylactic transfusion reactions are rare complications of blood transfusions. Anhaptoglobinemia, a condition that has high incidence in Asia, can cause allergic transfusion reactions or anaphylaxis in severe cases. A 50-yr-old Korean woman was diagnosed with relapsed acute promyelocytic leukemia. She developed thrombocytopenia during chemotherapy and an anaphylactic transfusion reaction on the 4th and 5th platelet transfusions immediately after the transfusion of the platelet concentrates was initiated. Blood analysis showed no detectable serum haptoglobin. We examined her genetic phenotype and detected anhaptoglobinemia, which occurs because of an allelic deletion in the Hp gene cluster. The presence of an antibody against haptoglobin was detected by performing ELISA. To prevent anaphylactic reactions, apheresis platelets were transfused after washing. Consequently, anaphylactic transfusion reactions did not develop. Here, we report the first case of anhaptoglobinemia causing anaphylactic transfusion reaction in Korea. PMID:22779074
Kim, Hyunsoo; Choi, Jonghyeon; Park, Kyoung Un; Kim, Hyon-Suk; Min, Yoo Hong; Kim, Moon Jung; Kim, Hyun Ok
Advances in safety of blood transfusion in clinical practice principally relate to preventing transfusion-transmitted infections (TTI). Epidemiological studies of TTI have resulted in the development, standardization, and implementation of an expanding array of immunoassays employed worldwide in routine screening of blood donated by voluntary blood donors. Exclusion of infected blood and their donors has remarkably reduced the risk of transmitting
Roger Dodd; W. Kurt Roth; Paul Ashford; Elizabeth M. Dax; Girish Vyas
Transfusion medicine has the logic of a therapeutic chain applied to labile blood components and cell therapy products, within a coherent structure, such as the recently created Etablissement français du sang. Faced to the threat of emerging--sometimes hypothetical--transfusion risks, such as the possible transmission of BSE by blood transfusion, the precaution principle requires developing strategies to reduce labile blood components consumption, by strictly defining the framework of blood transfusion prescription and encouraging the search for red blood cell and platelet substitutes. In the field of alternatives to labile blood components, research has however yielded few results. The future of transfusion medicine lies in biotechnology: cell (and gene) therapy will become part of novel therapeutic strategies for the treatment of numerous pathologies in man. Transfusion medicine will have to consider the significant advances achieved over the last few years in the field of multipotent stem cells. Transfusion medicine will thus find its place in the promising field of innovating therapies. PMID:11503508
Hervé, P; Tiberghien, P
Hemolysis is commonly seen in patients with mechanical heart valves and is secondary to destruction of red blood cells by mechanical action of artificial valve. It is very unusual after repair of native heart valve. Here we present a case of hemolytic anemia in association with mitral valve repair. PMID:23808095
Shailesh, Fnu; Deshmukh, Abhishek J; Singla, Sandeep
The inherited hemoglobin disorders sickle cell disease and thalassemia are the most common monogenetic disorders worldwide. Pulmonary hypertension is one of the leading causes of morbidity and mortality in adult patients with sickle cell disease and thalassemia, and hemolytic disorders are potentially among the most common causes of pulmonary hypertension. The pathogenesis of pulmonary hypertension in hemolytic disorders is likely multifactorial, including hemolysis, impaired nitric oxide (NO) bioavailability, chronic hypoxemia, chronic thromboembolic disease, chronic liver disease, and asplenia. In contrast to patients with traditional forms of pulmonary arterial hypertension, patients with hemolytic disorders have a mild-to-moderate degree of elevation in mean pulmonary pressures, with mild elevations in pulmonary vascular resistance. The hemodynamic etiology of pulmonary hypertension in these patients is multifactorial and includes pulmonary arterial hypertension, pulmonary venous hypertension, and pulmonary hypertension secondary to a hyperdynamic state. Currently, there are limited data on the effects of any specific treatment modality for pulmonary hypertension in patients with hemolytic disorders. It is likely that maximization of treatment of the primary hemoglobinopathy in all patients and treatment with selective pulmonary vasodilators and antiproliferative agents in patients with pulmonary arterial hypertension would be beneficial. However, there is still a major need for large multinational trials of novel therapies for this patient population.
Gladwin, Mark T.
Transfusion-related acute lung injury (TRALI) is a form of posttransfusion acute pulmonary insufficiency that has been linked to the infusion of biologic response modifiers (BRMs), including antileukocyte antibodies and lipids. Soluble CD40 ligand (sCD40L) is a platelet-derived proinflammatory mediator that accumulates during platelet storage. We hypothesized that human polymorpho-nuclear leukocytes (PMNs) express CD40, CD40 ligation rapidly primes PMNs, and sCD40L induces PMN-mediated cytotoxicity of human pulmonary microvascular endothelial cells (HMVECs). Levels of sCD40L were measured in blood components and in platelet concentrates (PCs) implicated in TRALI or control PCs that did not elicit a transfusion reaction. All blood components contained higher levels of sCD40L than fresh plasma, with apheresis PCs evidencing the highest concentration of sCD40L followed by PCs from whole blood, whole blood, and packed red blood cells (PRBCs). PCs implicated in TRALI reactions contained significantly higher sCD40L levels than control PCs. PMNs express functional CD40 on the plasma membrane, and recombinant sCD40L (10 ng/mL-1 ?g/mL) rapidly (5 minutes) primed the PMN oxidase. Soluble CD40L promoted PMN-mediated cytotoxicity of HMVECs as the second event in a 2-event in vitro model of TRALI. We concluded that sCD40L, which accumulates during blood component storage, has the capacity to activate adherent PMNs, causing endothelial damage and possibly TRALI in predisposed patients.
Khan, Samina Yasmin; Kelher, Marguerite R.; Heal, Joanna M.; Blumberg, Neil; Boshkov, Lynn K.; Phipps, Richard; Gettings, Kelly F.; McLaughlin, Nathan J.; Silliman, Christopher C.
Transfusion-related acute lung injury (TRALI) is a form of posttransfusion acute pulmonary insufficiency that has been linked to the infusion of biologic response modifiers (BRMs), including antileukocyte antibodies and lipids. Soluble CD40 ligand (sCD40L) is a platelet-derived proinflammatory mediator that accumulates during platelet storage. We hypothesized that human polymorpho-nuclear leukocytes (PMNs) express CD40, CD40 ligation rapidly primes PMNs, and sCD40L induces PMN-mediated cytotoxicity of human pulmonary microvascular endothelial cells (HMVECs). Levels of sCD40L were measured in blood components and in platelet concentrates (PCs) implicated in TRALI or control PCs that did not elicit a transfusion reaction. All blood components contained higher levels of sCD40L than fresh plasma, with apheresis PCs evidencing the highest concentration of sCD40L followed by PCs from whole blood, whole blood, and packed red blood cells (PRBCs). PCs implicated in TRALI reactions contained significantly higher sCD40L levels than control PCs. PMNs express functional CD40 on the plasma membrane, and recombinant sCD40L (10 ng/mL-1 mug/mL) rapidly (5 minutes) primed the PMN oxidase. Soluble CD40L promoted PMN-mediated cytotoxicity of HMVECs as the second event in a 2-event in vitro model of TRALI. We concluded that sCD40L, which accumulates during blood component storage, has the capacity to activate adherent PMNs, causing endothelial damage and possibly TRALI in predisposed patients. PMID:16772606
Khan, Samina Yasmin; Kelher, Marguerite R; Heal, Joanna M; Blumberg, Neil; Boshkov, Lynn K; Phipps, Richard; Gettings, Kelly F; McLaughlin, Nathan J; Silliman, Christopher C
Autologous blood transfusion techniques are the principal means of reducing allogeneic blood exposure. Those techniques were developed in order to prevent the risk of contamination by viruses, mainly HVB, HCV and HIV. However that risk has become so small that all studies show an exorbitant cost/efficiency ratio. Autologous blood transfusion would therefore be of no interest in terms of public health but a recent experimental study suggested a possible transmission of the BSE agent through blood. Until the matter is settled, the precaution principle means we should prefer alternative techniques to allogeneic blood whenever possible, hence a renewed interest in autologous transfusion. PMID:11503506
Rosencher, N; Conseiller, C
Although the risk of transfusion-transmitted infections today is lower than ever, the supply of safe blood products remains subject to contamination with known and yet to be identified human pathogens. Only continuous improvement and implementation of donor selection, sensitive screening tests and effective inactivation procedures can ensure the elimination, or at least reduction, of the risk of acquiring transfusion transmitted infections. In addition, ongoing education and up-to-date information regarding infectious agents that are potentially transmitted via blood components is necessary to promote the reporting of adverse events, an important component of transfusion transmitted disease surveillance. Thus, the collaboration of all parties involved in transfusion medicine, including national haemovigilance systems, is crucial for protecting a secure blood product supply from known and emerging blood-borne pathogens.
Bihl, Florian; Castelli, Damiano; Marincola, Francesco; Dodd, Roger Y; Brander, Christian
Text VersionDengue Transfusion Risk Model. Lyle R. Petersen, MD, MPH. ... D s = Duration of viremia before symptoms develop. Dengue Risk Model Variables. ... More results from www.fda.gov/downloads/advisorycommittees/committeesmeetingmaterials
\\u000a Blood transfusion is an effective and unmediated means of increasing the number of red blood cells in the circulation in order\\u000a to enhance athletic performance. Blood transfusion became popular in the 1970s among elite endurance athletes and declined\\u000a at the end of the 1980s with the introduction of recombinant erythropoietin. The successive implementation in 2001 of a direct\\u000a test to
Sylvain Giraud; Pierre-Edouard Sottas; Neil Robinson; Martial Saugy
Hereditary xerocytosis is a rare hemolytic anemia occurring secondary to a defect in cell membrane potassium flux. We report a case of severe fetal anemia and non-immune hydrops secondary to hereditary xerocytosis that was managed successfully with in utero erythrocyte and albumin transfusion. PMID:11483935
Ogburn, P L; Ramin, K D; Danilenko-Dixon, D; Fairbanks, V F; Ramsey, P S
Introduction Metformin is a widely prescribed biguanide antidiabetic drug that has been implicated as a cause of hemolytic anemia in three previous case reports. We report a case of rapidly fatal hemolysis that was temporally associated with the initiation of metformin treatment for diabetes. Clinicians need to be aware of this rare but potentially serious side effect of metformin. Case presentation A 56-year-old Caucasian man with type 2 diabetes mellitus was started on metformin to improve glycemic control. Shortly afterwards, he developed progressive fatigue, exertional dyspnea, cranberry-colored urine and jaundice. Laboratory studies showed severe hemolysis, with a drop in hemoglobin from 14.7 to 6.6 g/dl over 4 days, markedly elevated lactate dehydrogenase, bilirubin and reticulocyte counts, and a low haptoglobin level. A peripheral blood smear showed no schistocytes, and a direct Coombs test was positive for anti-IgG and negative for anti-C3. Despite corticosteroid treatment and transfusion of packed red blood cells, the patient developed increasing dyspnea, hypotension, further decline in hemoglobin to 3.3 g/dl, and fatal cardiorespiratory arrest 12 hours after admission. Conclusion The serologic findings in this case suggest an autoimmune hemolytic anemia, caused either by a drug-induced autoantibody or a warm autoantibody. Based on the temporal association with metformin and the lack of other clear precipitating causes, we propose that metformin-induced hemolysis with a drug-induced autoantibody is a strong possibility. This mechanism differs from a previously described case with a possible antibody to the erythrocyte-drug complex. It has been shown, however, that hemolysis may occur via multiple mechanisms from the same drug. Clinicians should consider the possibility of metformin-associated immune hemolytic anemia in patients with otherwise unexplained hemolysis.
Packer, Clifford D; Hornick, Thomas R; Augustine, Sarah A
Introduction Acquired haemophilia is a rare autoimmune disorder caused by autoantibodies directed in the majority of the cases against clotting factor VIII. This disorder is characterised by the sudden onset of bleeding that not rarely may be life-threatening and need transfusion support. Most reports on this condition describe the need for blood transfusions during the acute, haemorrhagic phase, but the number of transfused red cell units is often unknown. Patients and methods In the last 5 years, 14 patients with acquired haemophilia A were identified in the transfusion and haemophilia centres of Verona and Castelfranco Veneto. The transfusion support for these 14 patients was analyzed in this retrospective survey. Results The 14 patients required a total of 183 red cell units. The average transfusion requirement was 13 red cells units/patient, with a range from 0 to 38 units. Conclusions Eleven of the 14 patients studied needed strong transfusion support to enable any further management of the haemorrhages, as well as for eradication treatment of the autoantibodies to factor VIII. A relevant part of the management of haemorrhagic symptoms as well as the first choice for any further treatment (bleeding or the cure of the underlying disease) is transfusion of red blood cells.
Tagariello, Giuseppe; Sartori, Roberto; Radossi, Paolo; Risato, Renzo; Roveroni, Giovanni; Tassinari, Cristina; Giuffrida, Annachiara; Gandini, Giorgio; Franchini, Massimo
Advances in surgery and the extension of the surgical indications have amplified the problems due to hemorrhage, main side effect, and its treatment, which in the past consisted primarily in whole blood transfusion. Such practice, however, involves the surgeon in a series of problems due to shortage of blood donors, religious beliefs and most of all the risks related to the transfusion practice itself. Apart from early and late reactions, the risk of transmission of infective diseases, post-transfusional immunodepression and legal problems must be pointed out. Recently, to solve these problems, the indications to blood transfusion have been restricted to severe hypovolemic shock and severe untreatable hypoxia; the separate use of blood components has been privileged; and autologous blood transfusion techniques like pre-deposit for donation, normovolemic preoperative hemodilution and intraoperative autologous transfusions have been used. It's mandatory that the surgeon keeps in date with the Transfusional Medicine progresses working in strict collaboration with the Transfusional Services to best protect the good health of the patients he has in care. PMID:7547127
Catania, G; Petralia, G; Catalano, F; Marzullo, E
Summary Background The muscle-relaxing effects of succinylcholine are terminated via hydrolysis by plasma cholinesterase. There are multiple genetic variants of this enzyme and clinical circumstances that might influence the activity of plasma cholinesterase and eventually lead to prolonged neuromuscular blockade following succinylcholine application. Case Report Here, we report a parturient woman with atonic bleeding who suffered significant blood loss (hemoglobin 6.0 g·dL?1). For surgical curettage, general anesthesia was performed by using short-acting succinylcholine. By the end of the 105-minute procedure, the patient’s trachea was extubated. After extubation she showed signs of the prolonged neuromuscular blocking action of succinylcholine. At this time, the patient received an AB0-compatible red blood cell transfusion and recovered instantly from neuromuscular blockade. The plasma cholinesterase (3.200 U·L?1) was below the normal range (4.900–12.000 U·L?1). Patient’s blood DNA analysis revealed heterozygously the genetic K variant of plasma cholinesterase. After red blood cell transfusion, serum potassium was elevated (5.7 mmol·L?1; 4.4 mmol·L?1 prior to transfusion). Conclusions Pregnancy, blood loss and genetic variation contributed to impairment of plasma cholinesterase. Due to high-speed red blood cell transfusion, hemolytic release of erythrocyte cholinesterase might have terminated the neuromuscular blocking succinylcholine effect.
Eckle, Veit-Simon; Schmid, Eckhard; Fehm, Tanja; Grasshoff, Christian
Text Version... Process for Transfusion Related Fatalities and Donation Related Deaths, http://www.fda ... we could not rule out the transfusion as the cause of death. ... More results from www.fda.gov/downloads/biologicsbloodvaccines/safetyavailability
Individuals with glucose 6-phosphate dehydrogenase (G6PD) deficiency are at risk for the development of hemolytic anemia when given 8-aminoquinolines (8-AQs), an important class of antimalarial/antiinfective therapeutics. However, there is no suitable animal model that can predict the clinical hemolytic potential of drugs. We developed and validated a human (hu)RBC-SCID mouse model by giving nonobese diabetic/SCID mice daily transfusions of huRBCs from G6PD-deficient donors. Treatment of SCID mice engrafted with G6PD-deficient huRBCs with primaquine, an 8-AQ, resulted in a dose-dependent selective loss of huRBCs. To validate the specificity of this model, we tested known nonhemolytic antimalarial drugs: mefloquine, chloroquine, doxycycline, and pyrimethamine. No significant loss of G6PD-deficient huRBCs was observed. Treatment with drugs known to cause hemolytic toxicity (pamaquine, sitamaquine, tafenoquine, and dapsone) resulted in loss of G6PD-deficient huRBCs comparable to primaquine. This mouse model provides an important tool to test drugs for their potential to cause hemolytic toxicity in G6PD-deficient populations. PMID:24101478
Rochford, Rosemary; Ohrt, Colin; Baresel, Paul C; Campo, Brice; Sampath, Aruna; Magill, Alan J; Tekwani, Babu L; Walker, Larry A
Background. Donor plasma proteins are associated with non-hemolytic allergic reactions, such as urticaria or laryngeal edema, in platelet-transfusion recipients. Replacement of plasma with synthetic media from platelet concentrates (PCs) is considered to be effective in preventing such reactions. However, platelets preserved in media depleted of less than 10% plasma are reported to have functions inferior to those preserved in plasma.Methods.
Takeshi Yuasa; Hitoshi Ohto; Akira Suzuki; Fumio Shishido
Although the hemoglobin level of 100 g\\/L has been used for many years as the allogeneic red blood cell (RBC) transfusion trigger, current evidence indicates that for most patients a more restrictive transfusion strategy is at least as effective as and possibly superior to a liberal transfusion strategy. Moreover, the available data indicate that the use of smaller volumes of allogeneic
M. A Blajchman; P. C Hébert
Transfusion of labile blood products (LBPs) generates occasional inflammatory : type, hazards; for a large part of these, no antigen/antibody conflict is thus, detected. Residual leucocytes used to account for a large part of such incidents - rarely accidents. Since, however, the systematic leukoreduction of LBPs, leucocytes are the less and less incriminated in adverse events. Platelets themselves proved capable of secreting copious amounts of inflammatory mediators, even in the absence of any deliberated stimulation. Meanwhile, even though exceptionally, inflammation can be observed after red blood cell transfusion. It has been noticed that the collection mode of cellular compounds, as well as the preparation and storage conditions are capable of inflicting lesions to the cell membranes and to activate those cells, and thus promoting inflammatory responses. Storage lesions as well as ageing of the stored cells alongside with cell apoptosis contribute to inflammatory responses. This present 'State of the Art' paper aims at encompassing the primary and secondary components of the LBPs, along with the various types of molecules displaying pro-inflammatory properties that can be encountered in transfusion. A better knowledge of causes of inflammatory transfusion-linked hazards is indeed instrumental to the implementation of safety measures aimed at reducing or suppressing these unwanted effects. PMID:23587611
Garraud, O; Cognasse, F; Hamzeh-Cognasse, H; Laradi, S; Pozzetto, B; Muller, J-Y
Summary This paper summarizes in an overview the new possibilities based on biotechnology like monoclonal antibodies and genetic engineering. Examples are given where these new techniques are already used in transfusion work. In addition some perspectives for the future in relation to these techniques are given.
Researchers have reviewed the role of blood transfusions in renal and marrow graft recipients. Striking contrasts are evident: while transfusions may promote successful kidney grafting, any transfusions before initiation of the transplant conditioning regimen may jeopardize the treatment of severe aplastic anemia by marrow transplantation. Researchers have suggested guidelines for the transfusion support of transplant candidates before transplantation and for marrow graft recipients after transplantation. It is important to recognize that after conditioning for marrow transplantation, all patients will be profoundly pancytopenic for a limited period of time, and intensive transfusion support is vital to patient survival.
Storb, R.; Weiden, P.L.
[Emergence of donor-derived anti-HLA antibody and subsequent transfusion-refractory thrombocytopenia after allogeneic hematopoietic stem cell transplantation from an HLA-matched sibling donor in a patient with acute myeloid leukemia].
A 45-year-old woman with acute myelogenous leukemia developed platelet transfusion refractoriness (PTR) after the engraftment of an allogeneic peripheral blood stem cell transplantation (PBSCT) from her multiparous sister, which was attributed to HLA antibodies that could not be detected in the patient's serum before transplantation. She achieved neutrophil engraftment by day 18 and megakaryocytopoiesis and complete donor chimerism was confirmed in the bone marrow on day 21. IgG-class HLA antibodies were detected in her serum on day 24 after PBSCT; however, on day 15, no HLA antibodies were detected. The specificity of the antibodies that emerged in the patient closely resembled that of the antibodies found in the donor. The donor had probably been immunized during pregnancy by their partner's HLA-antigens expressed by the fetus. Consequently, transplanted donor-derived cells provoked HLA antibodies in the recipient early after PBSCT, and those HLA antibodies induced PTR. The presence of HLA antibodies should be examined at least in pregnant female donors whose recipients developed PTR attributable to HLA antibodies after SCT. PMID:23470830
Uchiyama, Yuri; Hoshino, Takumi; Mihara, Masahiro; Mitsui, Takeki; Koiso, Hiromi; Takizawa, Makiko; Yokohama, Akihiko; Saitoh, Takayuki; Uchiumi, Hideki; Handa, Hiroshi; Tsukamoto, Norifumi; Murakami, Hirokazu; Nojima, Yoshihisa
The ?-globin gene mutation in sickle cell anemia results in anemia and repeated bouts of vascular occlusion. The cumulative effect of these vasocclusive events is progressive damage to many organs including the kidneys, lungs, and brain. The transfusion of red blood cells (RBC) can ameliorate many of these complications, but can be associated with both acute and chronic complications, including iron overload. The objective of the Best Practices in Transfusion Medicine for Patients with Sickle Cell Disease (SCD) Conference was to review the available published evidence and clinical experience surrounding the use of RBC transfusions for sickle cell disease by a panel of experts. The expert panel developed explicit clinical guidelines for the use of RBC in SCD patients. The panel also made recommendations for further research. A set of guidelines were produced for dissemination to pertinent stakeholders. If implemented, these clinical pathways have the potential to optimize the use of red blood cell transfusions in SCD.
Wun, Ted; Hassell, Kathryn
The physiological anaemia experienced by preterm babies is exacerbated by common care practices such as early clamping of the umbilical cord at birth and gradual exsanguination by phlebotomy for laboratory monitoring. The need for subsequent transfusion with red blood cells can be reduced by delaying cord clamping for 30–60 s in infants who do not require immediate resuscitation. The need for transfusions can be further reduced by limiting phlebotomy losses, providing good nutrition, and using standard guidelines for transfusion based on haemoglobin or haematocrit. What those guidelines should be is not clear. Analysis of two recent large clinical trials comparing restrictive and liberal transfusion guidelines leads to several conclusions. Restrictive transfusion guidelines may reduce the number of transfusions given, but there is no reduction in donor exposures if a single-donor transfusion programme is used. There is some evidence that more liberal transfusion guidelines may help to prevent brain injury, but information on the impact of transfusion practice on long-term outcome is lacking. Until further guidance emerges, transfusion thresholds lower than those used in the two trials should not be used, as there is no evidence that lower thresholds are safe.
Hemolytic uremic syndrome is the leading cause of acute kidney injury in childhood. Ninety percent of cases are secondary to gastrointestinal infection with shigatoxin-producing bacteria. In this review, we discuss the molecular mechanisms of shigatoxin leading to hemolytic uremic syndrome and the emerging role of the complement system and vascular endothelial growth factor in its pathogenesis. We also review the evidence for treatment options to date, in particular antibiotics, plasma exchange, and immunoadsorption, and link this to the molecular pathology. Finally, we discuss future avenues of treatment, including shigatoxin-binding agents and complement inhibitors, such as eculizumab.
Keir, Lindsay S; Marks, Stephen D; Kim, Jon Jin
The serogroups A (Streptococcus pyogenes), B (Streptococcus agalactiae) and S. dysgalactiae subsp. equisimilis (group C and G) are generally defined as beta-hemolytic streptococci. Beta-hemolytic streptococci cause a variety of infections ranging from skin and soft-tissue infections to severe invasive infections such as necrotizing fasciitis (NF) and streptococcal toxic shock syndrome (STSS). The case fatality rate due to bacteremias caused by beta-hemolytic streptococci is 15%. The use of clindamycin in combination with benzylpenicillin has been shown to be of benefit. The use of intravenous immunoglobulin is suggested in STSS in combination with antibiotics and surgery. PMID:23961606
Sickle cell disease is a hereditary pathology of the haemoglobin which affects only individuals from African ancestry. The frequency of the disease increases in France. Transfusion remains a major treatment of this disease. Depending of the indication, transfusion can be a simple transfusion or an exchange transfusion. In this last case, exchange can be performed manually or automatically. The transfusion protocols have to be adapted to the polytransfused status of these patients, but also to the high incidence of alloimmunisation against red blood cells. Alloimmunisation is a consequence of the polymorphism of blood groups between sickle cell disease patients and donors of European ancestry. Axes to optimize transfusion safety in these patients have to be developed. But the first step relies on the promotion of blood donation within individuals of African ancestry. PMID:23597585
The New Guideline for Transfusion Practice (TP) and the New Criteria for Indication of Blood Products for Transfusion (BPfT) were revised in September 2005. Both are based on the New Blood Law approved in July 2003, which defines the principles of TP and determines the responsibility of the related parties. The New Blood Law and the revised guidelines require safe and appropriate TP; namely, avoid mismatched transfusion, determine indication appropriately and supply sufficiently safe blood products to cover the domestic demand. The New Guideline for TP defines the essential points of the hospital management system for appropriate TP. The establishment of a practical management system based on active hospital transfusion committee (HTC) advice is essential. The establishment of a transfusion service equipped for laboratory testing and unification of the management system of TP; a medical doctor responsible for TP with full commitment to transfusion management, a transfusion manual, specialized laboratory technicians and a 24-hour system for transfusion testing and provision are required. Autologous blood transfusion should be recommended as the safest TP, when appropriate management and operation systems are established. The New Criteria for the Indication of BPfT show the summarized criteria for the indication of each blood product at the beginning, and detailed information about each field at the end. Also described are the inappropriate use of each blood product, the trigger level and the objective level of blood components, the timing, dosage and rate of transfusion for each product, the transfusion-related record database, and the evaluation of transfusion effectiveness from the viewpoint of improvement of the clinical status as well as the laboratory data. PMID:17265897
Acute rhabdomyolysis results from susceptible persons eating quail during the migrating season. The etiology is unknown. Muscular exercise is an important precipitating factor. In this paper the literature on this and related rhabdomyolytic and hemolytic ...
J. B. Bateman
This article reports a case of hypotensive reaction following platelet transfusion (PT) and presents a possible etiologic mechanism implicating negatively charged leukocyte reduction filters (LRFs) and angiotensin converting enzyme (ACE) inhibitors. A 14-year-old boy with acute lymphoblastic leukemia was admitted to the pediatric intensive care unit (PICU) for respiratory failure following bone marrow transplantation. He was being treated with ACE
France Gauvin; Baruch Toledano; Heather A. Hume; Jacques Lacroix
Shiga toxin-producing Escherichia coli (STEC) hemolytic uremic syndrome (HUS) is an important cause of acute kidney injury (AKI). The outcomes of STEC HUS have improved, and the acute mortality rate in children is 1-4 %. About 70 % of patients recover completely from the acute episode and the remainder have varying degrees of sequelae. Only a few retrospective studies have reviewed these patients over long periods. Methodological flaws include a lack of strict definitions, changing modes of treatment, ascertainment bias and loss of subjects to follow-up. The kidneys bear the brunt of the long-term damage: proteinuria (15-30 % of cases); hypertension (5-15 %); chronic kidney disease (CKD; 9-18 %); and end-stage kidney disease (ESKD; 3 %). A smaller number have extra-renal sequelae: colonic strictures, cholelithiasis, diabetes mellitus or brain injury. Most renal sequelae are minor abnormalities, such as treatable hypertension and/or variable proteinuria. Most of the patients who progress to ESKD do not recover normal renal function after the acute episode. Length of anuria (more than 10 days) and prolonged dialysis are the most important risk factors for a poor acute and long-term renal outcome. After the acute episode all patients must be followed for at least 5 years, and severely affected patients should be followed indefinitely if there is proteinuria, hypertension or a reduced glomerular filtration rate (GFR). PMID:23288350
Spinale, Joann M; Ruebner, Rebecca L; Copelovitch, Lawrence; Kaplan, Bernard S
Autoimmune Hemolytic Anemia (AIHA), a very infrequent condition which represents a group of disorders in which presence of autoantibodies directed against self-antigens leads to shortened red cell survival. Till date, a very few cases of AIHA in Malaria patients are reported worldwide but still AIHA should be considered a relatively rare cause of anemia in malaria. A 20 year male presented with intermittent fever since seven days and yellowish discoloration of urine and sclera since 5 days. He was transfused three units of blood at a private clinic before one month. On examination, pallor, icterus and spelnomegaly were present. Hemoglobin (Hb) was 3.2 gm% and peripheral smear revealed ring forms of both Plasmodium vivax and Plasmodium falciparum. Serum LDH and Serum billirubin (Indirect and Direct) were high. This patient’s blood group was B +ve with positive autocontrol. Indirect Antiglobulin Test (IAT), antibody screening and antibody identification were pan-positive with reaction strength of +4 against each cell. Direct Antiglobulin Test was +4 positive anti IgG and negative with anti C3. He was treated with Artesunate and methylprednisone. Least incompatible, saline washed O Neg and B neg red cells were transfused on the 2nd day of starting treatment. Hb was raised to 6.1 gm% on 4th day. Patient was discharged on 9th day with Hb 7.0 gm% with oral tapering dose of steroids. In the above case, patient was suffering from high grade malarial parasitemia with co-existing autoimmune RBC destruction by IgG auto-antibodies which led to sudden drop in Hb and rise in serum LDH and indirect billirubin. Least incompatible packed red cells along with antimalarials and steroids led to clinical improvement. So far, one case report each from India, Korea, Canada and Germany and one case series report of three cases from India have been reported. Under-reporting or rarity of this phenomenon may be accountable for this.
Sonani, Rajesh; Bhatnagar, Nidhi; Maitrey, Gajjar
Autoimmune Hemolytic Anemia (AIHA), a very infrequent condition which represents a group of disorders in which presence of autoantibodies directed against self-antigens leads to shortened red cell survival. Till date, a very few cases of AIHA in Malaria patients are reported worldwide but still AIHA should be considered a relatively rare cause of anemia in malaria. A 20 year male presented with intermittent fever since seven days and yellowish discoloration of urine and sclera since 5 days. He was transfused three units of blood at a private clinic before one month. On examination, pallor, icterus and spelnomegaly were present. Hemoglobin (Hb) was 3.2 gm% and peripheral smear revealed ring forms of both Plasmodium vivax and Plasmodium falciparum. Serum LDH and Serum billirubin (Indirect and Direct) were high. This patient's blood group was B +ve with positive autocontrol. Indirect Antiglobulin Test (IAT), antibody screening and antibody identification were pan-positive with reaction strength of +4 against each cell. Direct Antiglobulin Test was +4 positive anti IgG and negative with anti C3. He was treated with Artesunate and methylprednisone. Least incompatible, saline washed O Neg and B neg red cells were transfused on the 2(nd) day of starting treatment. Hb was raised to 6.1 gm% on 4(th) day. Patient was discharged on 9th day with Hb 7.0 gm% with oral tapering dose of steroids. In the above case, patient was suffering from high grade malarial parasitemia with co-existing autoimmune RBC destruction by IgG auto-antibodies which led to sudden drop in Hb and rise in serum LDH and indirect billirubin. Least incompatible packed red cells along with antimalarials and steroids led to clinical improvement. So far, one case report each from India, Korea, Canada and Germany and one case series report of three cases from India have been reported. Under-reporting or rarity of this phenomenon may be accountable for this. PMID:24014948
Sonani, Rajesh; Bhatnagar, Nidhi; Maitrey, Gajjar
Objective: Maternal allo-antibody production is stimulated when fetal red blood cells are positive for an antigen absent on the mother's red cells. The maternal IgG antibodies produced will pass through the placenta and attack fetal red cells carrying the corresponding antigen. Allo-immune hemolytic disease of the fetus and newborn caused by anti-E rarely occurs. Case summary: We report two cases of anti-E hemolytic diseases in neonates. One of the neonates had severe hemolysis presenting with severe anemia, thrombocytopenia, and conjugated hyperbilirubinemia, while the other had moderate anemia and unconjugated hyperbilrubinemia. Although both the neonates were treated by phototherapy and intravenous immunoglobulin, one of them received double volume exchange transfusion. Conclusion: There appeared to be an increase in the occurrence of hemolytic disease of the fetus and newborn caused by Rh antibodies other than anti-D. In this case report, both patients presented with anemia and hyperbilirubinemia but were successfully treated, with a favorable outcome.
Usman, Adiyyatu Sa'idu; Mustaffa, Rapiaah; Ramli, Noraida; Diggi, Sirajo A.
The Mayo Clinic, in Rochester, Minnesota, recently set forth a directive to develop a Mayo Emergency Incident Command System (MEICS) plan to respond to major disasters. The MEICS plan that was developed interfaces with national response plans to ensure effective communication and coordination between our institution and local, state, and federal agencies to establish a common language and communication structure. The MEICS plan addresses multiple aspects of dealing with resource needs during a crisis, including the need for blood and transfusion medicine services. The MEICS plan was developed to supplement our current local emergency preparedness procedures and provide a mechanism for responding to the escalating severity of an emergency to deal with situations of a magnitude that is outside the normal experience. A plan was developed to interface the existing Transfusion Medicine disaster plan standard operating procedures (SOP) with the institutional and Department of Laboratory Medicine (DLMP) MEICS plans. The first step in developing this interface was defining MEICS. Other major steps were defining the chain of command, developing a method for visually indicating who is "in charge," planning communication, defining the actions to be taken, assessing resource needs, developing flowcharts and updating SOPs, and developing a blood rationing team to deal with anticipated blood shortages. Several key features of the interface and updated disaster plan that were developed are calling trees for response personnel, plans for relocating leadership to alternative command centers, and action sheets to assist with resource assessment. The action sheets also provide documentation of key actions by response personnel. PMID:19845076
Bundy, K L; Foss, M L; Stubbs, J R
Summary In 2 cases of RhD-sensitized women, hemolytic disease of the newborns (hdn) was mitigated by intrauterine transfusions of·Rh-negative donor blood during pregnancy. In both cases preterm delivery was performed by cesarean section in week 32 of pregnancy. With cord blood the direct antiglobulin test (DAT) was negative using the gel test. The blood groups corresponded to the transfused donor
R. Lynen; T. J. Legler; J. H. Maas; A. Suren; R. Osmers; B. Behring; W. Schroeter; M. Koehler
The incidence of sepsis caused by transfusion of bacterially contaminated blood components is similar to or less than that of transfusion-transmitted hepatitis C virus infection, yet significantly exceeds those currently estimated for transfusion-associated human immunodeficiency and hepatitis B viruses. Outcomes are serious and may be fatal. In addition, transfusion of sterile allogenic blood can have generalized immunosuppressive effects on recipients, resulting in increased susceptibility to postoperative infection. This review examines the frequency of occurrence of transfusion-associated sepsis, the organisms implicated, and potential sources of bacteria. Approaches to minimize the frequency of sepsis are discussed, including the benefits and disadvantages of altering the storage conditions for blood. In addition, the impact of high levels of bacteria on the gross characteristics of erythrocyte and platelet concentrates is described. The potentials and limitations of current tests for detecting bacteria in blood are also discussed.
Wagner, S J; Friedman, L I; Dodd, R Y
Blood donation is an act of solidarity. Most often, this act is done on a volunteer basis and, depending on countries and circumstances, is not remunerated. The increase in need, the always-greater number of deferral criteria, the safety issues and the changes in the structures of our societies are among the many subjects for ethical debates. Taking these into account, the actors of the transfusion must analyze certain parameters: the value of a donation, the meaning of volunteering, the appropriateness of remunerating the act of giving a part of one's self, no longer as a donation or an expression of altruism and solidarity, but as a commercial act regimented by economic laws. PMID:23916572
Tissot, J-D; Garraud, O; Danic, B; Cabaud, J-J; Lefrère, J-J
One of transfusion's side effects is alloimmunization against red blood cell (RBC) antigens. Early diagnosis by antibody screening is an important step in the detection of these alloantibodies. The authors studied the frequency of alloimmunization in thalassemic patients of 4 centers (2 adult and 2 pediatric centers) and compared the rates in children (up to 15 years) and adults. Antibody screening tests were performed by gel method according to its standard pattern and respective program. In positive cases, antibody identification test by gel method was performed. Eight hundred thirty-five patients were studied; 548 (65.6%) were adults (mean age = 24.5), and 287 (34.4%) cases were pediatrics (mean age = 10.05). Of these patients, 74.1% had no history of transfusion reaction, whereas 21 (2.5%) had hemolytic complications. Seventy-eight (9.3%) exhibited allergic symptoms, and 117 (14%) cases experienced febrile reactions during transfusion. Antibody screening showed positive results in 22 pediatric cases (7.7%) and 79 adults (14.4%); 72 (71.3%), 19 (18.8%), 3 (3%), and 1 (1%) cases exhibited single, double, triple, and autoantibodies, respectively. Anti-Kell antibody was seen in 34 (33.7%) cases, anti-D was seen in 11 (10.9%) cases, and anti-E in was seen in 10 (9.9%) cases. The authors observed 8 anti-D+C (7.9%) cases, 1 anti-D+E (1%), 3 anti-Kell+E, 3 anti-Kell+Kpa (3%), and 1 anti-Kell+D double antibodies. These antibodies were also a combination of Rh subgroups or Rh and Kell subgroups. The authors observed meaningful relations between history of transfusion reactions and age with antibody screening results (P = .005). Based on alloantibodies types, more than two thirds of them were Rh subgroups and Kell groups. Phenotype determination of RBCs before beginning chronic blood transfusion and careful cross-matching with Kell and Rh subgroups in addition to ABO may help reduce alloimmunization in chronic transfusion patients. PMID:21854216
Azarkeivan, Azita; Ansari, Shahla; Ahmadi, Mohammad Hossein; Hajibeigy, Bashir; Maghsudlu, Mahtab; Nasizadeh, Soheila; Shaigan, Mojgan; Toolabi, Abdolmajid; Salahmand, Mitra
Thirty thrombocytopaenic patients of acute leukaemias and myelodysplastic syndrome were transfused platelets collected from ABO-matched donors using Haemonetics V30 and V50 blood processors. Twenty-seven patients had septicaemia and/or splenomegaly; 2 patients had disseminated intravascular coagulation (DIC). Pre-transfusion platelet count was 11.0 +/- 4.0 X 10(9)/L. The mean corrected count increments (CCI) 1 hour and 18 hours post-transfusion were 13.02 X 10(9)/L and 3.88 X 10(9)/L respectively, in the absence of DIC. Active bleeding stopped when platelet count was above 15.0 X 10(9)/L. There was no difference between the platelet yield from two blood processors. PMID:2768161
Charak, B S; Rao, K; Parikh, P M; Rawat, R S; Nair, C N; Advani, S H
“When should we trigger a transfusion?” is always a critical question between the patient's benefits and risks in RBC transfusion. A computerized transfusion decision support system (CTDSS) has been used since September 2004 in an academic medical center with 1400 beds. In this study, the factors affecting red blood cell (RBC) transfusion were investigated. Totally 20,551 RBC-transfusion episodes between January
Chao-Sung Chang; Yu-Chih Lin; Chiu Chu Lin; Chi-Jung Yeh; Yung-Chao Wu; Yi-Ching Lin
Autoimmune hemolytic anemia is rare in children and infants and steroids are the corner stone of therapy. Management of the patients with steroid refractory/dependent disease is difficult .Rituximab is being used in the treatment of a variety of autoimmune diseases including Autoimmune hemolytic anemia (AIHA),especially in adults but there is scarce data regarding the use of this agent in pediatric AIHA patients.The authors report two cases of steroid refractory AIHA, who responded to rituximab with review the literature of its use in pediatrics. PMID:21830023
Gupta, Nitin; Sharma, Sanjeev; Seth, Tulika; Mishra, Pravas; Mahapatra, Manoranjan; Kumar, Suman; Kapoor, Rajan; Agarwal, Narendra
... Department of Health and Human Services Public Health Service Food and Drug Administration 1401 Rockville Pike Rockville, MD 20852-1448. ... More results from www.fda.gov/biologicsbloodvaccines/safetyavailability/bloodsafety
This article reviews the use of transfusion medicine in veterinary medicine and discusses current research regarding donor screening and component therapy. Typing and crossmatching methodologies are discussed. Available components, potential uses, and controversies in treatment are also discussed. PMID:23747258
Hemolytic uremic syndrome (HUS) can be associated with different infectious etiologies, but the relationship between pseudomembranous colitis and HUS was first described in the 1970s in some childhood patients. There is very limited published literature on Clostridium difficile-associated HUS. We report a case of C. difficile-related HUS in an adult patient and provide a review of the literature.
Keshtkar-Jahromi, Maryam; Mohebtash, Mahsa
The clinical diagnosis of microangiopathic hemolytic anemia (MHA) can be ascertained only by the prove of fragmentocytes (schistocytes) in the peripheral blood. Those fragmented red cells caused by different processes of microangiopathy are partly destroyed in the blood vessels, partly absorbed by the reticuloendothelial system and may produce typical histomorphological changes. Therefore, besides the histological classification of the microangiopathy in
P. J. Klein; H. E. Schaefer; Féaux de Lacroix; R. Fischer
Atypical hemolytic uremic syndrome (aHUS) associated with membranoproliferative glomerulonephritis (MPGN) is an uncommon clinical presentation, especially in children. We report a 8-year-old-boy who presented like aHUS but the kidney biopsy showed MPGN type 1. PMID:24036644
Mehta, Kumud; More, Vaishali; Chitale, Arun; Khubchandani, Shaila
Biermer's disease is an autoimmune atrophic gastritis of the fundus predominantly responsible for a malabsorption of vitamin B12. Despite its association with several autoimmune disorders, few observations have reported an association with autoimmune hemolytic anemia (AIHA). We report a case of Biermer's disease associated with AIHA in a patient of 66 years old. PMID:22796620
Nafil, Hatim; Tazi, Illias; Mahmal, Lahoucine
Blood transfusions are a common therapeutic procedure in small animal medicine and have been investigated in some exotic species but little information is available about their safety and efficacy in reptiles. In human pediatrics and small animal practice, the Hemo-Nate18-micro filter is used to prevent embolic clots and particulate waste from entering the recipient during a transfusion. The goal of this study was to determine the hemolytic effect of an 18-micro Hemo-Nate filter for whole blood cell transfusions in reptiles using the American alligator (Alligator mississippiensis) as a reptilian model. Results revealed no significant difference in free plasma hemoglobin between the unfiltered and filtered samples (P = 0.21). There was no difference in the prefiltration and postfiltration packed cell volume (PCV) (P = 0.41). Results suggest that an 18-micro Hemo-Nate filter does not cause hemolysis or decrease the PCV of small quantities of alligator blood. PMID:22946400
Nevarez, Javier G; Cockburn, Jennifer; Kearney, Michael T; Mayer, Joerg
Massive bleeding in trauma patients is a serious challenge for all clinicians, and an interdisciplinary diagnostic and therapeutic approach is warranted within a limited time frame. Massive transfusion usually is defined as the transfusion of more than 10 units of packed red blood cells (RBCs) within 24 h or a corresponding blood loss of more than 1- to 1.5-fold of the body's entire blood volume. Especially male trauma patients experience this life-threatening condition within their productive years of life. An important parameter for clinical outcome is to succeed in stopping the bleeding preferentially within the first 12 h of hospital admission. Additional coagulopathy in the initial phase is induced by trauma itself and aggravated by consumption and dilution of clotting factors. Although different aspects have to be taken into consideration when viewing at bleedings induced by trauma compared to those caused by major surgery, the basic strategy is similar. Here, we will focus on trauma-induced massive hemorrhage. Currently there are no definite, worldwide accepted algorithms for blood transfusion and strategies for optimal coagulation management. There is increasing evidence that a higher ratio of plasma and RBCs (e.g. 1:1) endorsed by platelet transfusion might result in a superior survival of patients at risk for trauma-induced coagulopathy. Several strategies have been evolved in the military environment, although not all strategies should be transferred unproven to civilian practice, e.g. the transfusion of whole blood. Several agents have been proposed to support the restoration of coagulation. Some have been used for years without any doubt on their benefit-to-risk profile, whereas great enthusiasm of other products has been discouraged by inefficacy in terms of blood transfusion requirements and mortality or significant severe side effects. This review surveys current literature on fluid resuscitation, blood transfusion, and hemostatic agents currently used during massive hemorrhage in order to optimize patients' blood and coagulation management in emergency medical aid. PMID:22670125
Meißner, Andreas; Schlenke, Peter
Introduction Mixed warm and cold autoimmune hemolytic anemia runs a chronic course with severe intermittent exacerbations. Therapeutic options for the treatment of hemolysis associated with autoimmune hemolytic anemia are limited. There have been only two reported cases of the effective use of rituximab in the treatment of patients with mixed autoimmune hemolytic anemia. We report a case of severe mixed autoimmune hemolytic anemia that did not respond to steroids and responded to four weekly doses of rituximab (one cycle). Case presentation A 62-year-old Caucasian man presented with dyspnea, jaundice and splenomegaly. His blood work revealed severe anemia (hemoglobin, 4.9 g/dL) with biochemical evidence of hemolysis. Exposure to cold led to worsening of the patient's hemolysis and hemoglobinuria. A direct antiglobulin test was positive for immunoglobulin G and complement C3d, and cold agglutinins of immunoglobulin M type were detected. A bone marrow biopsy revealed erythroid hyperplasia. A positron emission tomographic scan showed no sites of pathologic uptake. There was no other evidence of a lymphoid or myeloid disorder. Initial therapy consisted of avoidance of cold, intravenous methylprednisolone and a trial of plasmapheresis. However, there was no clinically significant response, and the patient continued to be transfusion-dependent. He was then started on 375 mg/m2/week intravenous rituximab therapy. After two treatments, his hemoglobin stabilized and the transfusion requirement diminished. Rituximab was continued for a total of four weeks and led to the complete resolution of his hemolytic anemia and associated symptoms. At the patient's last visit, about two years after the initial rituximab treatment, he continued to be in complete remission. Conclusion To the best of our knowledge, this is the first reported case of mixed-type autoimmune hemolytic anemia that did not respond to steroid therapy but responded completely to only one cycle of rituximab. The previous two reports of rituximab use in mixed autoimmune hemolytic anemia described an initial brief response to steroids and the use of rituximab at the time of relapse. In both of these case reports, the response to one cycle of rituximab was short-lived and a second cycle of rituximab was required. Our case report demonstrates that severe hemolysis associated with mixed autoimmune hemolytic anemia can be unresponsive to steroid therapy and that a single cycle of rituximab may lead to prompt and durable complete remission.
Stroke is a relatively frequent and severe complication of sickle cell disease. We performed cerebral arteriograms in 30 patients with sickle cell disease to evaluate the cause of acute neurologic deficits and to assess the effects of transfusion therapy given for a year or more after the acute episode. Twenty-three patients with motor and speech deficits had multiple abnormalities of
Marie Olivieri Russell; Herbert I. Goldberg; Andrew Hodson; Haewon C. Kim; Joanne Halus; Martin Reivich; Elias Schwartz
Volume replacement is critical to the resuscitation of the hemorrhaging patient, but this usually can be accomplished quickly and safely with crystalloid and/or colloid solutions. Red cells should be used in addition to asanguinous fluids in the treatment of tissue hypoxia due to anemia. The need for whole blood as opposed to packed red blood cells is controversial. However, plasma should not be used as a volume expander, and its use to supplement coagulation factors during the massive transfusion of red cells should be guided by laboratory tests that document a coagulopathy. Similarly, platelet transfusions are indicated to correct documented thrombocytopenia or platelet dysfunction, and routine prophylaxis after fixed volumes of red cells results is unwarranted. Many anticipated complications of massive transfusions, including hemostatic abnormalities, acid-base imbalances, hyperkalemia, and hypocalcemia, are uncommon or of limited clinical significance. The risks of immune hemolysis and transfusion-transmitted diseases, on the other hand, are significant, and argue for judicious use of blood components. In emergencies in which blood is required immediately before compatibility testing can be completed, O-negative uncrossmatched blood can be requested. Careful blood specimen collection and patient identification prior to transfusion are critical. Practices that emphasize blood conservation, including the use of autologous salvaged blood, are always to the patient's advantage. PMID:3281521
Kruskall, M S; Mintz, P D; Bergin, J J; Johnston, M F; Klein, H G; Miller, J D; Rutman, R; Silberstein, L
Non-transfusion-dependent thalassemias include a variety of phenotypes that, unlike patients with beta (?)-thalassemia major, do not require regular transfusion therapy for survival. The most commonly investigated forms are ?-thalassemia intermedia, hemoglobin E/?-thalassemia, and ?-thalassemia intermedia (hemoglobin H disease). However, transfusion-independence in such patients is not without side effects. Ineffective erythropoiesis and peripheral hemolysis, the hallmarks of disease process, lead to a variety of subsequent pathophysiologies including iron overload and hypercoagulability that ultimately lead to a number of serious clinical morbidities. Thus, prompt and accurate diagnosis of non-transfusion-dependent thalassemia is essential to ensure early intervention. Although several management options are currently available, the need to develop more novel therapeutics is justified by recent advances in our understanding of the mechanisms of disease. Such efforts require wide international collaboration, especially since non-transfusion-dependent thalassemias are no longer bound to low- and middle-income countries but have spread to large multiethnic cities in Europe and the Americas due to continued migration.
Musallam, Khaled M.; Rivella, Stefano; Vichinsky, Elliott; Rachmilewitz, Eliezer A.
Alloimmunization against the Rhesus-D (RhD) antigen still remains as a major cause of hemolytic disease of fetus and newborn (HDFN). Determination of paternal RhDzygosity is performed by molecular testing and is valuable for the management of alloimmunized pregnant women. A 30-year-old pregnant woman with AB negative blood group presented with two consecutive abortions and no history of blood transfusion. By application of the antibody screening, identification panel, and selected cells, she was found to be highly alloimmunized. RhDzygosity was performed on her partner and was shown to be homozygous for RhD. The sequence- specific priming-polymerase chain reaction used in this report is essential to establish whether the mother requires an appropriate immunoprophylaxis or the fetus is at risk of HDFN. PMID:24014950
Moghaddam, Mostafa; Naghi, Amirali; Hassani, Fatemeh; Amini, Sedighe
Autoimmune hemolytic anemia (AIHA) is an immune mediated destruction of erythrocytes, which has a good prognosis in children. It is known to have chronic, remitting or relapsing course, especially in infants and adolescents. Treatment of refractory or relapsing AIHA is a challenge as the other aim of the treatment is to avoid prolonged exposure to steroids or other immunosuppressants in small children. Rituximab is used in patients who are non-responsive to conventional treatment such as steroids, intravenous immunoglobulins and transfusion therapy. It has varying therapeutic success rate. We report a case of AIHA in a 4-month-old infant who had ill-sustained response to conventional therapy, but responded to rituximab.
Makadia, Darshak; Siddaiahgari, Sirisha Rani; Latha, M. S.
... development of red blood cell antibodies, and iron overload in different organs of the body. Blood transfusion ... finds donors that match it very closely. Iron overload Iron overload occurs following frequent blood transfusions. All ...
Bone marrow replacement therapy following whole-body x- or gamma-irradiation has until now proven to be of limited value in the treatment of individuals with hemolytic disease. The large doses of radiation required for destruction of defective erythropoietic tissues coupled with their resultant high mortality appears to limit its usefulness. Techniques have been developed by the authors to limit the extent of exposure and to improve survival following irradiation. These techniques include shielding of all parts of the body except the hind limbs, prophylactic use of antibiotics, and preparatory blood transfusion to suppress the development of indigenous defective erythrocytes. Using these combined techniques we were able to establish high rates of survival, successful engraftment, and long-term clinical improvement in mice with several hemolytic disorders emanating from hereditary defects in spectrin production and incorporation. Evidence is presented indicating that complete bone marrow replacement occurs even in nonirradiated portions of the erythron and that only donor type red blood cells appear in the circulation.
Bernstein, S.E.; Deveau, S.A. (Jackson Lab., Bar Harbor, ME (USA))
We report a case of M. pneumoniae infection presenting with severe hemolytic anemia in a 4-year-old girl, with a ten-day history of paleness, weakness, and nonproductive cough. She was very pale and tachycardic. However, she was not tachypneic. Chest examination showed normal breath sounds. No rhoncus or whistling was heard. As the erythrocyte sedimentation rate was excessively elevated, the differential diagnosis primarily comprised hematological malignancies. Direct Coombs' test was positive. Diagnosis of M. pneumoniae infection was confirmed by elevated levels of M. pneumoniae IgG and IgM antibodies and a chest X-ray suggestive of atypical pneumonia. The patient was treated with clarithromycin and packed red cell transfusion and showed a favorable recovery within ten days after admission. In conclusion, this case demonstrates that severe hemolytic anemia caused by M. pneumoniae is not always associated with severe pulmonary involvement, even when the respiratory infection is very mild, M. pneumoniae may be the cause of severe anemia.
Kurugol, Zafer; Onen, Serife Sebnem; Koturoglu, Guldane
This paper presents evidence of a significantly enhanced humoral immune response to sheep red cells following thermal injury, as assayed by direct hemolytic antibody plaque formation. An increase in the number of 19 S IgM antibody-secreting cells from the spleen was observed in rats up to 16 days after a 30-percent total body surface area, third-degree, scald burn. Since this
R. F. Mortensen; K. Eurenius
Introduction. In the first 48 hours of ventilating patients with acute lung injury (ALI)/acute respiratory distress syndrome (ARDS), a multipronged approach including packed red blood cell (PRBC) transfusion is undertaken to maintain oxygen delivery. Hypothesis. We hypothesized children with ALI/ARDS transfused within 48 hours of initiating mechanical ventilation would have worse outcome. The course of 34 transfused patients was retrospectively compared to 45 nontransfused control patients admitted to the PICU at Helen DeVos Children's Hospital between January 1st 2008 and December 31st 2009. Results. Mean hemoglobin (Hb) prior to transfusion was 8.2?g/dl compared to 10.1?g/dl in control. P/F ratio decreased from 135.4 ± 7.5 to 116.5 ± 8.8 in transfused but increased from 148.0 ± 8.0 to 190.4 ± 17.8 (P < 0.001) in control. OI increased in the transfused from 11.7 ± 0.9 to 18.7 ± 1.6 but not in control. Ventilator days in the transfused were 15.6 ± 1.7 versus 9.5 ± 0.6 days in control (P < 0.001). There was a trend towards higher rates of MODS in transfused patients; 29.4% versus 17.7%, odds ratio 1.92, 95% CI; 0.6–5.6 Fisher exact P < 0.282. Conclusion. This study suggests that early transfusions of patients with ALI/ARDS were associated with increased ventilatory needs.
Rajasekaran, Surender; Sanfilippo, Dominic; Shoemaker, Allen; Curtis, Scott; Zuiderveen, Sandra; Ndika, Akunne; Stoiko, Michael; Hassan, Nabil
Bactericidal and hemolytic comple- ment activities were investigated in 51 quarter milk samples of 13 cows in late lactation. Hemolytic activity was in all of the samples but one, after accounting for whey inhibitory activity. Mean hemolytic activity and inhibitory activities were .18 and .34 complement hemolytic units. Inflammation, in relation to infection status, increased hemolytic titers and heat-labile bactericidal
P. Rainard; B. Poutrel; J. P. Caffin
The purpose of the study is to determine if cytomegalovirus (CMV) is transmitted from blood donors to blood recipients in the course of transfusion. One hundred-thirty pediatric patients undergoing open-heart surgery and 46 renal transplant recipients wer...
H. Monto J. A. Armstrong
The purpose of this study was to examine some aspects of umbilical cord blood collection for autologous transfusion in premature infants. All 120 microbacterial cultures (aerobic and anaerobic) of cord blood samples as well as 30 cultures of mycoplasma were treated. Cord prothrombin fragment (F 1 + 2) concentrations were quantified at one and 10 minutes after clamping of the
A. Ballin; E. Arbel; G. Kenet; M. Berar; D. Kohelet; A. Tanay; H. Zakut; D. Meytes
Common variable immunodeficiency is a primary immunodeficiency disease characterized by reduced serum immunoglobulins and heterogeneous clinical features. Recurrent pyogenic infections of upper and lower respiratory tracts are the main clinical manifestations of common variable immunodeficiency. Hemolytic uremic syndrome is a multisystemic disorder characterized by thrombocytopenia, microangiopathic hemolytic anemia, and organ ischemia due to platelet aggregation in the arterial microvasculature. This is one of the rare cases of patients diagnosed with common variable immunodeficiency, which was complicated by hemolytic uremic syndrome.
Red cell transfusions are associated with the development of acute lung injury in the critically ill. Recent evidence suggests that storage induced alterations of the red blood cell (RBC) collectively termed the “storage lesion” may be linked with adverse biologic consequences. Using a 2-event model of systemic endotoxemia followed by a secondary challenge of RBC transfusion, we investigated whether purified RBC concentrates from syngeneic C57BL/6 mice altered inflammatory responses in murine lungs. Transfusion of RBCs stored for 10 days increased neutrophil counts, macrophage inflammatory protein-2 (MIP-2) and chemokine (KC) concentrations in the airspaces, and lung microvascular permeability compared with transfusion of less than 1-day-old RBCs. Because RBCs have been shown to scavenge inflammatory chemokines through the blood group Duffy antigen, we investigated the expression and function of Duffy during storage. In banked human RBCs, both Duffy expression and chemokine scavenging function were reduced with increasing duration of storage. Transfusion of Duffy knockout RBCs into Duffy wild-type en-dotoxemic mice increased airspace neutrophils, inflammatory cytokine concentrations, and lung microvascular permeability compared with transfusion of Duffy wild-type RBCs. Thus, reduction in erythrocyte chemokine scavenging is one functional consequence of the storage lesion by which RBC transfusion can augment existing lung inflammation.
Mangalmurti, Nilam S.; Xiong, Zeyu; Hulver, Mei; Ranganathan, Mrunalini; Liu, Xiang Hong; Oriss, Timothy; Fitzpatrick, Meghan; Rubin, Marc; Triulzi, Darrell; Choi, Augustine
A prospective observational study was carried out over a seven month period in the neonatal intensive care unit (NICU) of a large Malaysian maternity hospital to determine the rate of blood transfusion and the incidence of transfusion reactions in newborn infants. During the study period, the rates of blood transfusion was 6.1% (n = 117) of NICU admission or 8.2 per 1,000 live births. The median birth weight of the infants who had received blood transfusion was 1,740 grams (range: 725-4,350), and their mean gestational age was 33.6 weeks (sd = 5.1, range = 24-41 weeks). The median age of infants when they first received blood transfusion was 4.0 days (range: 1-27 days). When compared with infants of birth weight between 3,000 and 3,499 grams, infants of birth weight less than 1,500 grams received significantly higher median number of transfusions per infant, (p < 0.001). The incidence of transfusion reaction was 2.7% (3/110) of all transfused infants or 1.3% (3/223) of all blood transfusions. Febrile nonhemolytic reaction was the only type of transfusion reaction detected during the study period. This study showed that transfusion reactions in newborn infants were not common. PMID:10971978
Boo, N Y; Chan, B H
Background Primary postpartum haemorrhage is an obstetrical emergency often causing acute anaemia that may require immediate red blood cell (RBC) transfusion. This anaemia results in symptoms such as fatigue, which may have major impact on the health-related quality of life. RBC transfusion is generally thought to alleviate these undesirable effects although it may cause transfusion reactions. Moreover, the postpartum haemoglobin level seems to influence fatigue only for a short period of time. At present, there are no strict transfusion criteria for this specific indication, resulting in a wide variation in postpartum policy of RBC transfusion in the Netherlands. Methods/Design The WOMB trial is a multicentre randomised non-inferiority trial. Women with acute anaemia due to postpartum haemorrhage, 12-24 hours after delivery and not initially treated with RBC transfusion, are eligible for randomisation. Patients with severe physical complaints are excluded. Patients are randomised for either RBC transfusion or expectant management. Health related quality of life (HRQoL) will be assessed at inclusion, at three days and one, three and six weeks postpartum with three validated measures (Multi-dimensional Fatigue Inventory, ShortForm-36, EuroQol-5D). Primary outcome of the study is physical fatigue three days postpartum. Secondary outcome measures are general and mental fatigue scores and generic health related quality of life scores, the number of RBC transfusions, length of hospital stay, complications and health-care costs. The primary analysis will be by intention-to-treat. The various longitudinal scores will be evaluated using Repeated Measurements ANOVA. A costs benefit analysis will also be performed. The power calculation is based on the exclusion of a difference in means of 1.3 points or greater in favour of RBC transfusion arm regarding physical fatigue subscale. With missing data not exceeding 20%, 250 patients per arm have to be randomised (one-sided alpha = 0.025, power = 80%). Discussion This study will provide evidence for a guideline regarding RBC transfusion in the postpartum patient suffering from acute anaemia. Equivalence in fatigue score, remaining HRQoL scores and physical complications between both groups is assumed, in which case an expectant management would be preferred to minimise transfusion reactions and costs. Trial registration ClinicalTrials.gov NCT00335023, Nederlands Trial Register NTR335
Introduction Blood transfusion is associated with increased morbidity and mortality in cardiac surgery patients, but cause-and-effect relations remain unknown. We hypothesized that blood transfusion is associated with changes in pulmonary and systemic inflammation and coagulation occurring in patients who do not meet the clinical diagnosis of transfusion-related acute lung injury (TRALI). Methods We performed a case control study in a mixed medical-surgical intensive care unit of a university hospital in the Netherlands. Cardiac surgery patients (n = 45) were grouped as follows: those who received no transfusion, those who received a restrictive transfusion (one two units of blood) or those who received multiple transfusions (at least five units of blood). Nondirected bronchoalveolar lavage fluid (BALF) and blood were obtained within 3 hours postoperatively. Normal distributed data were analyzed using analysis of variance and Dunnett's post hoc test. Nonparametric data were analyzed using the Kruskal-Wallis and Mann-Whitney U tests. Results Restrictive transfusion increased BALF levels of interleukin (IL)-1? and D-dimer compared to nontransfused controls (P < 0.05 for all), and IL-1? levels were further enhanced by multiple transfusions (P < 0.01). BALF levels of IL-8, tumor necrosis factor ? (TNF?) and thrombin-antithrombin complex (TATc) were increased after multiple transfusions (P < 0.01, P < 0.001 and P < 0.01, respectively) compared to nontransfused controls, but not after restrictive transfusions. Restrictive transfusions were associated with increased pulmonary levels of plasminogen activator inhibitor 1 compared to nontransfused controls with a further increase after multiple transfusions (P < 0.001). Concomitantly, levels of plasminogen activator activity (PAA%) were lower (P < 0.001), indicating impaired fibrinolysis. In the systemic compartment, transfusion was associated with a significant increase in levels of TNF?, TATc and PAA% (P < 0.05). Conclusions Transfusion during cardiac surgery is associated with activation of inflammation and coagulation in the pulmonary compartment of patients who do not meet TRALI criteria, an effect that was partly dose-dependent, suggesting transfusion as a mediator of acute lung injury. These pulmonary changes were accompanied by systemic derangement of coagulation.
Despite bacterial culture of platelets, transfusion-associated bacteremia/sepsis (TABS) may occur with a frequency of approximately 1/60,000 platelet transfusions, while an emerging transfusion-transmitted infection (TTI) could reproduce the epidemic of transfusion-transmitted human immunodeficiency virus (HIV) in the future. As platelet pathogen-reduction (PR) systems licensed in Europe may eventually become licensed in the U.S., three alternative strategies for reducing the residual risks of TTIs and TABS may become available in the U.S. in the future: (1) transfusion of (already-available) non-pathogen-reduced single-donor (as opposed to pooled whole-blood-derived [PWBD]) platelets, (2) transfusion of pathogen-reduced single-donor platelets, or (3) transfusion of pathogen-reduced PWBD platelets (if trials of this component are conducted in the U.S. in the future). PR of platelets will increase the risk of mild and moderate (albeit perhaps not severe) bleeding complications and it cannot protect from all pathogens. Compared to PWBD platelets, single-donor platelets can reduce, by at least twofold, the risk of all known and emerging TTIs, as well as the risk of TABS, without incurring any risk. The fewer donor exposures secured by the use of single-donor platelets - especially if combined with collection of red blood cells and/or plasma from the same donation for transfusion to the same recipient through the use of multicomponent apheresis - may also reduce the risk of transfusion-related acute lung injury. To choose between pathogen-reduced and non-pathogen-reduced single-donor platelets, the increased risks of bleeding complications as well as other possible adverse events secondary to PR need to be quantified precisely and weighed against the competing risks of TABS and emerging TTIs. PMID:21403979
Vamvakas, Eleftherios C
Injured patients stress the transfusion service with frequent demands for uncrossmatched red cells and plasma, occasional requirements for large amounts of blood products and the need for new and better blood products. Transfusion services stress trauma centers with demands for strict accountability for individual blood component units and adherence to indications in a clinical field where research has been difficult, and guidance opinion-based. New data suggest that the most severely injured patients arrive at the trauma center already coagulopathic and that these patients benefit from prompt, specific, corrective treatment. This research is clarifying trauma system requirements for new blood products and blood-product usage patterns, but the inability to obtain informed consent from severely injured patients remains an obstacle to further research.
Murthi, Sarah B; Dutton, Richard P; Edelman, Bennett B; Scalea, Thomas M; Hess, John R
The purpose of this study was to examine some aspects of umbilical cord blood collection for autologous transfusion in premature infants. All 120 microbacterial cultures (aerobic and anaerobic) of cord blood samples as well as 30 cultures of mycoplasma were treated. Cord prothrombin fragment (F 1 + 2) concentrations were quantified at one and 10 minutes after clamping of the cord. F 1 + 2 concentrations assessed on 25 newborn infants were similar and no linear association with time of clamping could be drawn. This means that cord blood thrombosis is not activated for at least 10 minutes following clamping of the cord. As far as is known, the first newborn infant to benefit from this method of transfusion is reported here. The premature infant received two portions of autologous blood (on days 5 and 7). No untoward effects were noted. Blood, collected from the umbilical cord, is a safe source for autotransfusion, provided that bacteriological testing has been carried out.
Ballin, A.; Arbel, E.; Kenet, G.; Berar, M.; Kohelet, D.; Tanay, A.; Zakut, H.; Meytes, D.
Non-Shiga-like toxin-producing Escherichia coli (STEC) or atypical hemolytic uremic syndrome (aHUS) is observed in 5-10% of all hemolytic uremic syndrome (HUS) cases, and usually develops secondary to infections, malignancies, drugs, transplantation, pregnancy, and autoimmune disease. However, there has been no report on adult onset HUS initiated by surgical procedures except transplantation. We report a 66-year-old woman who incurred renal impairment on the first day after laparoscopic hemicolectomy. Hemolytic anemia, thrombocytopenia, absence of Shiga toxin associated disease, normal ADAMTS13 activity, and low serum C3 (not C4) were consistent with a diagnosis of aHUS. We performed plasma exchange with fresh frozen plasma. Nevertheless, deteriorated renal function was not recovered after the treatment. Although it is an uncommon postoperative complication, aHUS needs to be considered as a possible cause of acute kidney injury combined with thrombocytopenia and anemia after surgical procedures, considering its different treatment modality and poor outcomes. PMID:23560430
Lee, Jae-Won; Won, Nam Hee; Cho, Eunjung; Kim, Myung-Gyu; Jo, Sang-Kyung; Cho, Won Yong; Kim, Hyoung Kyu
Thrombotic microangiopathy, hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura. The term thrombotic microangiopathy (TMA) defines a lesion of vessel wall thickening (mainly arterioles or capillaries), intraluminal platelet thrombosis, and partial or complete obstruction of the vessel lumina. Depending on whether renal or brain lesions prevail, two pathologically indistinguishable but somehow clinically different entities have been described: the hemolytic uremic syndrome
Piero Ruggenenti; Marina Noris; Giuseppe Remuzzi
The aim of this paper was to focus on the toxinological aspects of microbasic-mastigophore nematocysts isolated from acontia of Aiptasia mutabilis, an Anthozoan collected in the Strait of Messina, by performing hemolytic assay on human, chicken, and rabbit red blood cells in suspension. The hemolytic effects of single isolated nematocysts were achieved by checking the lytic pattern after discharge. Crude
A. Marino; G. Musci; G. La Spada
The inadvertent transfer of food allergy from an allergic donor to an unsuspecting recipient by transfusion or organ donation\\u000a is a relatively rare but intriguing event with potentially catastrophic consequences. Additionally, the development of food\\u000a allergy in the recipient of a transplant from a donor who was not food allergic poses questions about why this occurs, why\\u000a it is observed
Dan Atkins; Jonathan Malka-Rais
Current risk from transfusion is largely because of noninfectious hazards and defects in the overall process of delivering safe transfusion therapy. Safe transfusion therapy depends on a complex process that requires integration and coordination among multiple hospital services including laboratory medicine, nursing, anesthesia, surgery, clerical support, and transportation. The multidisciplinary hospital transfusion committee has been traditionally charged with oversight of transfusion safety. However, in recent years, this committee may have been neglected in many institutions. Resurgence in hospital oversight of patient safety and transfusion efficacy is an important strategy for change. A new position, the transfusion safety officer (TSO), has been developed in some nations to specifically identify, resolve, and monitor organizational weakness leading to unsafe transfusion practice. New technology is becoming increasingly available to improve the performance of sample labeling and the bedside clerical check. Several technology solutions are in various stages of development and include wireless handheld portable digital assistants, advanced bar coding, radiofrequency identification, and imbedded chip technology. Technology-based solutions for transfusion safety will depend on the larger issue of the technology for patient identification. Devices for transfusion safety hold exciting promise but need to undergo clinical trials to show effectiveness and ease of use. Technology solutions will likely require integration with delivery of pharmaceuticals to be financially acceptable to hospitals. PMID:12881778
Dzik, Walter H; Corwin, Howard; Goodnough, Lawrence Tim; Higgins, Martha; Kaplan, Harold; Murphy, Michael; Ness, Paul; Shulman, Ira A; Yomtovian, Rosyln
Warm autoimmune hemolytic anemia (WAIHA) is one of four clinical types of autoimmune hemolytic anemia (AIHA), with the characteristics of autoantibodies maximally active at body temperature. It produces a variable anemia—sometimes mild and sometimes severe. With respect to the absence or presence of an underlying condition, WAIHA is either idiopathic (primary) or secondary, which determines the treatment strategies in practice. Conventional treatments include immune suppression with corticosteroids and, in some cases, splenectomy. In recent years, the number of clinical studies with monoclonal antibodies and immunosuppressants in the treatment of WAIHA increased as the knowledge of autoimmunity mechanisms extended. This thread of developing new tools of treating WAIHA is well exemplified with the success in using anti-CD20 monoclonal antibody, Rituximab. Following this success, other treatment methods based on the immune mechanisms of WAIHA have emerged. We reviewed these newly developed immunotherapy treatments here in order to provide the clinicians with more options in selecting the best therapy for patients with WAIHA, hoping to stimulate researchers to find more novel immunotherapy strategies.
BACKGROUND The hemolytic–uremic syndrome consists of the triad of microangiopathic hemolytic anemia, thrombocytopenia, and renal failure. The common form of the syndrome is triggered by infection with Shiga toxin–producing bacteria and has a favorable outcome. The less common form of the syndrome, called atypical hemolytic–uremic syndrome, accounts for about 10% of cases, and patients with this form of the syndrome have a poor prognosis. Approximately half of the patients with atypical hemolytic–uremic syndrome have mutations in genes that regulate the complement system. Genetic factors in the remaining cases are unknown. We studied the role of thrombomodulin, an endothelial glycoprotein with anticoagulant, antiinflammatory, and cytoprotective properties, in atypical hemolytic–uremic syndrome. METHODS We sequenced the entire thrombomodulin gene (THBD) in 152 patients with atypical hemolytic–uremic syndrome and in 380 controls. Using purified proteins and cell-expression systems, we investigated whether thrombomodulin regulates the complement system, and we characterized the mechanisms. We evaluated the effects of thrombomodulin missense mutations associated with atypical hemolytic–uremic syndrome on complement activation by expressing thrombomodulin variants in cultured cells. RESULTS Of 152 patients with atypical hemolytic–uremic syndrome, 7 unrelated patients had six different heterozygous missense THBD mutations. In vitro, thrombomodulin binds to C3b and factor H (CFH) and negatively regulates complement by accelerating factor I–mediated inactivation of C3b in the presence of cofactors, CFH or C4b binding protein. By promoting activation of the plasma procarboxypeptidase B, thrombomodulin also accelerates the inactivation of anaphylatoxins C3a and C5a. Cultured cells expressing thrombomodulin variants associated with atypical hemolytic–uremic syndrome had diminished capacity to inactivate C3b and to activate procarboxypeptidase B and were thus less protected from activated complement. CONCLUSIONS Mutations that impair the function of thrombomodulin occur in about 5% of patients with atypical hemolytic–uremic syndrome.
Delvaeye, Mieke; Noris, Marina; De Vriese, Astrid; Esmon, Charles T.; Esmon, Naomi L.; Ferrell, Gary; Del-Favero, Jurgen; Plaisance, Stephane; Claes, Bart; Lambrechts, Diether; Zoja, Carla; Remuzzi, Giuseppe; Conway, Edward M.
The hemolytic uremic syndrome (HUS) is the end result of a variety of etiologic agents that can induce endothelial cell injury and thrombotic microangiopathy (TMA) mostly within the kidney. The typical, post-diarrheal verocytotoxin associated HUS (D + HUS) is the major cause of acute renal failure in children worldwide. In the course of HUS treatment, fluid overload is usually the result of overhydration in the context of oliguria or anuria which cause edema, hypertension, worsening of neurologic signs and cardiac failure. Appropriate and timely use of dialysis has dramatically reduced complications of renal failure and extra-renal complications are now the main causes of mortality and morbidity in D + HUS. The reasons for treatment by infusion of fresh frozen plasma and/or plasmapheresis for D + HUS are theoretical and their therapeutic effects are inconclusive. We believe that plasma administration for regular D + HUS has no value and is potentially harmful. Until new strategies become available in clinical practice, the general consensus for the moment is that careful supportive management with patience is still the most appropriate form of D + HUS therapy. PMID:9086786
Ito, K; Shiraga, H
Hemolyitic uremic syndrome (HUS), characterized by triad of acute kidney injury, thrombocytopenia, and hemolytic anemia, has considerable morbidity and mortality and is known to be associated with diarrheal illness. It usually occurs after a diarrheal illness due to Shiga-toxin-producing Escherichia coli. Streptococcus pneumoniae is a rare but well recognized trigger for nondiarrhea associated HUS in children, but has not been reported in adults. We report a case of an adult presenting with pneumococcal pneumonia complicated by HUS and required renal replacement therapy.Immunoglobulin M (IgM) nephropathy is an idiopathic glomerulonephritis characterized by mesangial deposits of IgM. IgM nephropathy presenting with proteinuria, especially nephrotic syndrome, frequently is steroid-dependent or steroidresistant and associated with reaching endstage renal disease after a 15-year follow-up. Because no long-term effective treatment is known for patients with IgM nephropathy, there is a clear need for therapeutic alternatives. We describe a patient with IgM nephropathy represented by recurrence of nephrotic syndrome who achieved longterm remission with interferon-? sustained treatment. PMID:23380391
Allen, Jennifer C; McCulloch, Thomas; Kolhe, Nitin V
The high-frequency Chido (Ch) antigen, found predominantly in plasma, is a determinant of the C4d fragment of the C4 molecule and is acquired by red cells during in vivo complement activation. Antibodies are made by Ch- people who lack C4S. It has often been reported that anti-Ch (and anti-Rg) do not cause hemolytic transfusion reactions. Reported here is a case of a transfusion reaction caused by anti-Ch. The antibody did not cause red cell destruction, but did cause a life-threatening anaphylactic reaction during transfusion of plasma proteins in pooled platelets. The antibody was of the IgG4 subclass and might have caused a short-term, sensitizing anaphylactic response. This case, and one previously reported in which a patient with anti-Rg experienced a severe reaction to fresh-frozen plasma and a plasma derivative, illustrates that these antibodies can cause severe, life-threatening reactions in patients who receive plasma-containing components. PMID:1502713
Westhoff, C M; Sipherd, B D; Wylie, D E; Toalson, L D
Audits of transfusion used as educational tools can improve transfusion practices. Effective audits must first identify problem(s) in transfusion practice and must then include as educational target, the attending physician. Educational methods that have been shown to he effective include: (1) meeting briefly one-on-one with physicians, (2) teaching at scheduled conferences, (3) making daily clinical rounds on patients who receive
Late-onset erythropoietic protoporphyria (EPP) is rare, and it is usually associated with an acquired somatic mutation of the ferrochelatase gene secondary to hematological malignancy such as myelodysplastic syndrome or myeloproliferative disorder. In 0.5–1% of patients with EPP, deposition of protoporphyrin in the liver leads to progressive liver insufficiency. Herein, we report the case of a 67-year-old female who developed EPP with typical photosensitivity and hemolytic anemia. Six months later, she was admitted with acute liver damage with a rapidly progressing course, and developed liver insufficiency. She recovered from the liver insufficiency after undergoing plasmapheresis and red blood cell exchange transfusion. A bone marrow examination revealed normal features; however, a cytogenetic analysis identified an abnormal clone of cells with a translocation between chromosomes 13q12 and 18q21.1. This is the first report of a patient who recovered from liver insufficiency. The results of this report suggest that plasmapheresis and red blood cell exchange transfusion are effective for treating liver insufficiency in patients with late-onset EPP.
Oshikawa, Yuka; Fukushima, Satoshi; Miyake, Taiga; Kawaguchi, Takeshi; Motomura, Kenta; Nakashima, Yasuhiro; Nakamura, Kenichi; Jinnin, Masatoshi; Ihn, Hironobu
... Vaccines, Blood & Biologics. ... International Society of Blood Transfusion (ISBT). Except where inconsistent with the regulations ... More results from www.fda.gov/biologicsbloodvaccines/developmentapprovalprocess/advertisinglabelingpromotionalmaterials
... Cerner Bridge Medical Transfusion Administration and Specimen Collections 3.3. Applicant: Cerner Corp, Kansas City MO. ... More results from www.fda.gov/biologicsbloodvaccines/bloodbloodproducts/approvedproducts
The purpose of this study was to characterize transfusion practices in the management of sickle cell disease and to identify factors attributing to differences in prescribing practices among Florida hematologists/oncologists. A cross-sectional study was performed in 2005-2006 utilizing a mail survey. The survey instrument addressed practice characteristics, sickle cell patient populations, transfusion settings, indications and techniques, red blood cell phenotype specifications/modifications, use of practice guidelines, and educational resource utilization. One hundred fifty two physicians (75% adult-oriented, 25% pediatric) completed the survey. Non-academic practice settings (78 %) were the primary location. Pediatric practices had a larger percentage of patients with overt strokes, and receiving hydroxyurea therapy than adult-oriented practices. The majority of survey respondents did not request limited phenotypically matched red blood cells on a routine basis. The majority of pediatric practices (60%) had individually defined transfusion practice guidelines in contrast to 8% of adult-oriented practices. There were statistically significant differences for pediatric and adult-oriented practices in managing certain acute and chronic transfusion indications. Analysis of clinical vignette data revealed variation among hematologists/oncologists in the transfusion management of common clinical scenarios. The study underscores the need for the development and dissemination of comprehensive sickle cell transfusion guidelines and protocols.
Dunbar, Levette N.; Coleman Brown, LaRae; Rivera, Donna R.; Hartzema, Abraham G.; Lottenberg, Richard
To improve the outcome of severe twin-to-twin transfusion syndrome with 1 hydropic fetus and to prevent ischemic sequelae in the survivor, we developed a technique of selective feticide by vascular embolization of the most severely damaged twin. Acute second trimester polyhydramnios occurred in 4 biamniotic monochorial twin pregnancies, with 1 fetus normal on ultrasound but the other severely damaged by
Marc Dommergues; Laurent Mandelbrot; Anne-Lyse Delezoide; Marie-Cécile Aubry; Laurent Fermont; Dominique Caputo-Mahieu; Yves Dumez
More than 90% of the Plesiomonas shigelloides strains that we tested produced a beta-hemolysin, as judged by the results of agar overlay and contact-dependent hemolysis assays. The hemolysin was cell associated, was active against the erythrocytes of various animal species, and was synthesized at both 25 and 35 degrees C. Activity was lost after thermal or proteolytic treatments or after preincubation in the presence of gentamicin; hemolytic activity did not appear to correlate with the previously established 50% lethal doses for seven of these strains. The hemolysin may play a role in iron acquisition in vivo via the lysis of erythrocytes, liberating hemoglobin, or, alternatively, may be involved in gastrointestinal disease.
Janda, J M; Abbott, S L
Purpose of review Complement mediated hemolytic uremic syndrome (aHUS) accounts for a significant proportion of non-shiga toxin HUS. The purpose of this review is to outline the pathophysiology, clinical features and therapeutic options for aHUS. Recent findings In the last decade, strides have been made in identifying several new disease-causing mutations in complement-regulating proteins. Summary Complement mediated HUS (aHUS) has a worse prognosis compared with shiga toxin mediated HUS, often resulting in end stage renal disease. Early identification of aHUS is crucial so that plasma therapy can be initiated. After renal transplantation, there is very high risk of disease recurrence and graft loss. Eculizumab and combined liver–kidney transplantation offer promise for improved prognosis.
Joseph, Catherine; Gattineni, Jyothsna
Atypical hemolytic uremic syndrome (aHUS) is a rare, lifethreatening, chronic, genetic disease of uncontrolled alternative pathway complement activation. The understanding of the pathophysiology and genetics of this disease has expanded over recent decades and promising new developments in the management of aHUS have emerged. Regardless of the cause of aHUS, with or without a demonstrated mutation or autoantibody, blockade of terminal complement activation through C5 is of high interest as a mechanism to ameliorate the disease. Eculizumab, an existing monoclonal antibody directed against C5 with high affinity, prevents the perpetuation of the downstream activation of the complement cascade and the damage caused by generation of the anaphylotoxin C5a and the membrane attack complex C5b-9, by blocking C5 cleavage. We report the successful use of eculizumab in a patient after kidney transplantation and discuss the disease aHUS. PMID:22517061
Hodgkins, Kavita S; Bobrowski, Amy E; Lane, Jerome C; Langman, Craig B
The risk from cumulative erythrocyte transfusions is poorly understood in oncology populations. This analysis among long-term survivors explored variation in transfusional burden over progressive eras of treatment identifying those at risk for iron overload. Transfusion records of 982 survivors of hematological malignancies treated at St. Jude were reviewed. After exclusions, 881 (90%) were assessed for cumulative volume, weight-adjusted volume and transfusion number. Treatment intensity was assigned using the Intensity of Treatment Rating Scale version 3.0 (ITR-3). Hematopoietic stem cell transplant and acute myeloid leukemia survivors had greater transfusional burden than conventional therapy recipients and acute lymphoblastic leukemia survivors, respectively. Survivors of 5-10 years were more likely than survivors of > 10 years to receive ? 10 transfusions (odds ratio = 2.0, 95% confidence interval 1.5-2.8). Those with higher ITR-3 scores and more recent decades of treatment had a higher transfusional burden. Comprehensive transfusion histories are useful in identifying those at highest risk for iron overload. PMID:23163568
Nottage, Kerri; Gurney, James G; Smeltzer, Matthew; Castellanos, Maria; Hudson, Melissa M; Hankins, Jane S
Lymphoid follicles evolve in the perivascular connective tissue of many organs, including the thymuses, in NZB/Bl mice with hemolytic anemia. In previously published studies, these thymic follicles have been held to be causal in the autoimmune genesis of the hemolytic anemia. The present study contradicts this interpretation by demonstrating: (a) lymphoid follicles develop in the perivascular connective tissue of many organs in NZB mice, and are not restricted to the thymuses; and (b) thymic lymphoid follicles develop in aged Swiss mice without hemolytic anemia. Contrary to previous reports, the thymuses of NZB mice contain normal numbers of Hassall's corpuscles, which develop from preexisting thymic epithelial cells, and not from blood vessels.
The -D- phenotype is a rare Rh phenotype that strongly expresses D antigen without C, c, E, or e antigens. In -D- phenotype individuals, anti-Rh17 (Hro) is commonly found if there is a history of pregnancy or transfusion with red blood cells (RBCs) that express C, c, E, or e antigens. We report the first case of a -D- phenotype patient with multiple Rh antibodies including anti-Rh17 who had a history of two occasions of transfusion with eight random donor platelet concentrates two and six years ago. We found that a trivial amount of RBCs in the platelet components was able to trigger sensitization to RBC antigens, especially the highly immunogenic and clinically significant Rh antigens, including C, c, E, e or CcEe polypeptides. To avoid unnecessary sensitization and to minimize the risk of hemolytic transfusion reactions in patients with this rare Rh phenotype, a modified strategy for pretransfusion screenings needs to be discussed in the field of transfusion medicine.
Yun, Jae Won; Kang, Eun-Suk; Ki, Chang-Seok; Koh, Kwang Cheol
Prion infections lead to invariably fatal diseases of the CNS, including Creutzfeldt-Jakob disease (CJD) in humans, bovine spongiform encephalopathy (BSE), and scrapie in sheep. There have been hundreds of instances in which prions have been transmitted iatrogenically among humans, usually through neurosurgical procedures or administration of pituitary tissue extracts. Prions have not generally been regarded as blood-borne infectious agents, and case-control studies have failed to identify CJD in transfusion recipients. Previous understanding was, however, questioned by reports of prion infections in three recipients of blood donated by individuals who subsequently developed variant CJD. On reflection, hematogenic prion transmission does not come as a surprise, as involvement of extracerebral compartments such as lymphoid organs and skeletal muscle is common in most prion infections, and prions have been recovered from the blood of rodents and sheep. Novel diagnostic strategies, which might include the use of surrogate markers of prion infection, along with prion removal strategies, might help to control the risk of iatrogenic prion spread through blood transfusions. PMID:16932576
Aguzzi, Adriano; Glatzel, Markus
Autologous blood transfusion is indicated and helpful for any patient who is entering surgery in whom the surgeon anticipates a large blood loss because of the multiple or combined plastic operations being planned for one surgical sitting. The advantages of autologous over homologous transfusions include avoidance of incompatibility reactions, better volume expansion, and no risk of acquiring diseases.
Carlos Oscar Uebel; Peter Pohl; Josué Bahlis
In 3 cases of severe twin transfusion syndrome we demonstrate that the concentration of atrial natriuretic factor (ANF) in the cord blood of recipient twins is significantly elevated compared to that of donor twins. The discrepancy between recipient and donor concentration correlates with the volume of transfusion. The following pathophysiological mechanism for explaining polyhydramnios in recipient twins is proposed: chronic
Peter Wieacker; Christian Wilhelm; Heinrich Prömpeler; Karl-Georg Petersen; Helmuth Schillinger; Meinert Breckwoldt
The development of new technologies leads to the discovery of new viruses. For each of these new infectious agents relevance to transfusion needs to be assessed. The questions to be answered are transmissibility by transfusion, pathogenicity, prevalence in blood donors, persistence and the availability of screening assays. Since 1995, three new viruses have been identified and extensively studied. GB virus-C\\/hepatitis
We report a case of transfusion-associated bacteremia caused by Psychrobacter arenosus. This psychrotolerant bacterium was previously isolated in 2004 from coastal sea ice and sediments in the Sea of Japan, but not from humans. P. arenosus should be considered a psychrotolerant bacterial species that can cause transfusion-transmitted bacterial infections. PMID:23764120
Caspar, Yvan; Recule, Christine; Pouzol, Patricia; Lafeuillade, Bruno; Mallaret, Marie-Reine; Maurin, Max; Croize, Jacques
The discovery of AIDS in the 1980s and its rapid evolution as a major concern for physicians and their patients have led to many questions about the safety of the blood supply. The attention placed on AIDS has led to new discoveries and technologies to reduce the risk of other transfusion complications such as hepatitis, bac- terial contamination, and transfusion-associated
Paul M. Ness
Fluorescein-labeled immunoglobulin G fractions from serums of patients with acute glomerulonephritis and from many normal serums stained the glomerular basement membrane and mesangium of renal tissue from patients with early acute glomerulonephritis; these serums did not stain the corresponding tissues from patients with any other kidney disease. Previous absorption of the serum fraction with frozen and thawed nephritogenic beta hemolytic
Gerhard Treser; Martin Semar; Melinda McVicar; Maxine Franklin; Antonia Ty; Inge Sagel; Kurt Lange
The discovery of citrate anticoagulant in the 1920s and the development of plastic packs for blood collection in the 1960s laid the groundwork for platelet transfusion therapy on a scale not previously possible. A major limitation, however, was the finding that platelet concentrates prepared from blood anticoagulated with citrate were unsuitable for transfusion because of platelet clumping. We found that this could be prevented by simply reducing the pH of platelet-rich plasma to about 6.5 prior to centrifugation. We used this approach to characterize platelet kinetics and sites of platelet sequestration in normal and pathologic states and to define the influence of variables such as anticoagulant and ABO incompatibility on post-transfusion platelet recovery. The "acidification" approach enabled much wider use of platelet transfusion therapy until alternative means of producing concentrates suitable for transfusion became available. PMID:24177466
Aster, Richard H
Pediatric hemolytic uremic syndrome (HUS) is a rare complication of infections usually caused by Escherichia coli; Streptococcus pneumoniae may be a causative agent in 5% of cases and is often more serious in terms of morbidity and mortality. We report a case of pediatric HUS following an infection by a serotype of S. pneumoniae not included in the vaccine administered to the child. Bacterial neuraminidase revealed a T-antigen and a Tk-antigen and red blood cells polyagglutinability in the laboratory test. Transfusion has been reoriented by an indication of secondary preparations: deplasmatisation of red blood cells and platelets and abstention of therapeutic plasma administration. HUS evolved favorably in a few days but the child retains consequences of meningitis cerebral anoxia. PMID:23587591
Rinaudo-Gaujous, Mélanie; Talagrand, Emilie; Verhoeven, Paul O; Garraud, Olivier; Flourié, Françoise
Text Version... The effects of cell-free, unbound Hb observed in hemolytic anemia, malaria, and SCD include high blood pressure in the arteries of the lung ... More results from www.fda.gov/downloads/biologicsbloodvaccines/scienceresearch
The triggers of secondary thrombotic thrombopcytopenic purpura (TTP) include drug toxicity, radiation and high-dose chemotherapy, angioinvasive infections, surgery and acute graft versus host disease. TTP secondary to surgery have been reported in a number of cases. Most of the cases have been occurred after open heart surgery. Extensive endothelial damage is held responsible as the initiating mechanism in postoperative TTP cases. However, there is no report of secondary TTP describing development owing to ABO incompatible blood transfusion. Here, we describe a patient who developed TTP after transfusion of ABO incompatible blood during hospitalization for bypass surgery. We also propose a hypothesis which may account for the possible underlying mechanism.
Solak, Yalcin; Selcuk, Nedim Yilmaz; Gaipov, Abduzhappar; Ucar, Ramazan; Biyik, Zeynep; Acar, Kadir
Acute Parvovirus B19 infection is responsible for blocking the erythroblastic line, usually with no consequences on hematopoiesis except in patients with chronic hemolytic anemia in whom it can evolve to potentially serious acute anemia. We report 2 observations of acute erythroblastopenia revealing hereditary spherocytosis in 2 children (1 boy and 1 girl) of non-consanguineous parents. PMID:21820287
Kamoun, T; Chabchoub, I; Aissa, K; Ben Mansour, L; Hachicha, M
We studied an infant with severe nonimmune hemolytic anemia and hydrops fetalis at birth. His neonatal course was marked by ongoing hemolysis of undetermined etiology requiring repeated erythrocyte transfusions. He has remained transfusion-dependent for more than 2 yr. A previous sibling born with hemolytic anemia and hydrops fetalis died on the second day of life. Peripheral blood smears from the parents revealed rare elliptocytes. Examination of their erythrocyte membranes revealed abnormal mechanical stability as well as structural and functional abnormalities in spectrin. Genetic studies revealed that the proband and his deceased sister were homozygous for a mutation of betaIsigma1 spectrin, L2025R, in a region of spectrin that is critical for normal function. The importance of leucine in this position of the proposed triple helical model of spectrin repeats is highlighted by its evolutionary conservation in all beta spectrins from Drosophila to humans. Molecular modeling demonstrated the disruption of hydrophobic interactions in the interior of the triple helix critical for spectrin function caused by the replacement of the hydrophobic, uncharged leucine by a hydrophilic, positively charged arginine. This mutation must also be expressed in the betaIsigma2 spectrin found in muscle, yet pathologic and immunohistochemical examination of skeletal muscle from the deceased sibling was unremarkable.
Gallagher, P G; Petruzzi, M J; Weed, S A; Zhang, Z; Marchesi, S L; Mohandas, N; Morrow, J S; Forget, B G
Twin-twin transfusion syndrome (TTTS) is a serious condition that affects 10% to 15% of twin pregnancies with monochorionic diamniotic placentation. The pathophysiology of TTTS is not completely understood; however, the presence of unbalanced placental vascular communications within a shared circulation has been implicated in its development. The presentation of TTTS is highly variable, and it does not always progress in a predictable manner. Monochorionic twin gestations should, therefore, be monitored for signs of TTTS with serial sonograms starting in the second trimester. Early TTTS can be managed conservatively. However, without intervention, early-onset advanced TTTS is associated with a high perinatal loss rate and risk of severe neurologic impairment among survivors. Limited studies suggest that fetoscopic laser photocoagulation is the best available treatment for advanced TTTS diagnosed in the second trimester. Even with laser therapy, there remains a significant risk of twin demise and neurologic handicap in survivors. PMID:22713499
Mosquera, Claudia; Miller, Russell S; Simpson, Lynn L
A 37 year old female with acute renal failure following malignant hypertension and microangiopathic hemolytic anemia as a side effect of oral contraceptives is reported as a first from Japan. Although she had enough medical treatment including hemodialysis for 2 years from the onset of symptoms, the complete remission did not occur due to severe narrowing of the small renal arteries. 3 interval biopsies revealed considerable myxoid intimal thickening of small arcuated and interlobular arteries without evident histological regression. No fibrinoid necrosis is present in arterioles. Glomeruli show a wrinkling of GBM with a partial mesangial interposition and a translucent subendothelial deposit. A year after onset, both kidneys contracted slightly. Platelet factors which were probably released during the hemolytic uremic state in the kidney may play an important role in severe myxoid intimal thickening of the small renal arteries. (author's) PMID:7328888
Fukushima, K; Yamagata, Y; Taguchi, T; Matsuo, K; Koga, N; Takebayashi, S
Pentoxifylline is an agent that improves red blood cell deformability (known as a hemorrheologic effect) and reduces blood viscosity. Here, we present a case of a patient with hemolytic anemia after continuous-flow left ventricular assist device (CF-LVAD) implantation that was successfully treated with pentoxifylline. Our case is a 64-year-old African American woman who was implanted with a HeartMate II device on August 6, 2010, as a bridge to transplant for end-stage heart failure. Her postoperative course was complicated by recurrent gastrointestinal bleeding and antiplatelet therapy was discontinued. On October 25, 2011, she was readmitted with anemia and hemoglobin of 6.6 mg/dl and no identifiable source of bleeding. Her lactate dehydrogenase (LDH) was 936 IU/L, indicating severe hemolysis. Due to her evidence of hemolytic anemia and her inability to tolerate antiplatelet therapy due to recurrent bleeding, she was discharged on pentoxifylline 400 mg thrice daily on October 27, 2011, with hemoglobin of 11.2 mg/dl after transfusion. After 60 days of pentoxifylline, her hemoglobin and LDH in clinic were 10.1 mg/dl and 223 IU/L, respectively. The patient was successfully bridged to transplant in June 2012. Additional analysis of pentoxifylline as a therapeutic modality to manage hemolytic anemia after CF-LVAD implantation may be warranted. PMID:23896772
Jennings, Douglas L; Williams, Celeste T; Morgan, Jeffrey A
It is now clearly established that the transfusion of blood from variant CJD (v-CJD) infected individuals can transmit the disease. Since the number of asymptomatic infected donors remains unresolved, inter-individual v-CJD transmission through blood and blood derived products is a major public health concern. Current risk assessments for transmission of v-CJD by blood and blood derived products by transfusion rely on infectious titers measured in rodent models of Transmissible Spongiform Encephalopathies (TSE) using intra-cerebral (IC) inoculation of blood components. To address the biological relevance of this approach, we compared the efficiency of TSE transmission by blood and blood components when administrated either through transfusion in sheep or by intra-cerebral inoculation (IC) in transgenic mice (tg338) over-expressing ovine PrP. Transfusion of 200 µL of blood from asymptomatic infected donor sheep transmitted prion disease with 100% efficiency thereby displaying greater virulence than the transfusion of 200 mL of normal blood spiked with brain homogenate material containing 103ID50 as measured by intracerebral inoculation of tg338 mice (ID50 IC in tg338). This was consistent with a whole blood titer greater than 103.6 ID50 IC in tg338 per mL. However, when the same blood samples were assayed by IC inoculation into tg338 the infectious titers were less than 32 ID per mL. Whereas the transfusion of crude plasma to sheep transmitted the disease with limited efficacy, White Blood Cells (WBC) displayed a similar ability to whole blood to infect recipients. Strikingly, fixation of WBC with paraformaldehyde did not affect the infectivity titer as measured in tg338 but dramatically impaired disease transmission by transfusion in sheep. These results demonstrate that TSE transmission by blood transfusion can be highly efficient and that this efficiency is more dependent on the viability of transfused cells than the level of infectivity measured by IC inoculation.
Andreoletti, Olivier; Litaise, Claire; Simmons, Hugh; Corbiere, Fabien; Lugan, Severine; Costes, Pierrette; Schelcher, Francois; Vilette, Didier; Grassi, Jacques; Lacroux, Caroline
Beta (?)-thalassemia is characterized by a hypercoagulable state and an increased risk of thrombosis, which can result in significant morbidity and mortality. The molecular and cellular mechanisms contributing to hypercoagulability are diverse and include chronic platelet activation, alteration of red blood cell membranes, abnormal expression of adhesion molecules on vascular endothelial cells, and dysregulation of hemostasis. Regular transfusions decrease the risk of thrombosis, whereas splenectomy significantly increases the risk. Splenectomized adults with non-transfusion-dependent thalassemia are also at high risk for ischemic brain damage. Strategies to lower the risk of thrombosis should be considered, including transfusion therapy to raise hemoglobin levels and avoidance or delay of splenectomy. PMID:22631037
Cappellini, Maria D; Musallam, Khaled M; Poggiali, Erika; Taher, Ali T
|Presents a primer for health educators about blood donation and transfusion, examining the nature of human blood, the background of blood transfusion, blood donation criteria, risks related to homologous blood transfusion, directed blood donation, potential alternatives to homologous transfusion, and resources for education on the subject. (SM)|
Felts, W. Michael; Glascoff, Mary A.
Objectives: Very low birth weight (<1500 g) infants frequently require packed red blood cell transfusions, and transfusion rates vary among neonatal intensive care units (NICUs). We analyzed transfusions and compared outcomes among NICUs. Study design: In a 6-site prospective study, we abstracted all newborns weighing <1500 g (total = 825) born between October 1994 and September 1995. Transfusion frequency and
Francis J. Bednarek; Stuart Weisberger; Douglas K. Richardson; Ivan D. Frantz; Bhavesh Shah; Lewis P. Rubin
Careful assessment of risks and benefits has to precede each decision on allogeneic red blood cell (RBC) transfusion. Currently, a number of key issues in transfusion medicine are highly con- troversial, most importantly the influence of different transfusion thresholds on clinical outcome. The aim of this article is to review current evidence on blood transfusions, to highlight 'hot topics' with
C. Madjdpour; D. R. Spahn
Many preterm infants are given multiple red blood cell transfusions during the early weeks of life. Because firm standards for neonatal transfusions do not exist, it is important to consider the pathophysiology of the anemia of prematurity, the goals of transfusion therapy and blood banking practices that best provide safe and effective neonatal transfusions. There is increasing agreement that efforts
Ronald G. Strauss
Background context: The risks and costs of transfusion are a great concern in the area of pediatric spine surgery, because it is a blood-intensive procedure with a high risk for transfusion. Therefore, determining the predictors of transfusion in this patient population is an important first step and has the potential to improve upon the current approaches to reducing transfusion rates.
Michael G. Vitale; Douglas E. Levy; Maxwell C. Park; Hyunok Choi; Julie C. Choe; David P. Roye
Labile blood products contain phosphatidylserine-expressing cell dusts, including apoptotic cells and microparticles. These cell by-products are produced during blood product process or storage and derived from the cells of interest that exert a therapeutic effect (red blood cells or platelets). Alternatively, phosphatidylserine-expressing cell dusts may also derived from contaminating cells, such as leukocytes, or may be already present in plasma, such as platelet-derived microparticles. These cell by-products present in labile blood products can be responsible for transfusion-induced immunomodulation leading to either transfusion-related acute lung injury (TRALI) or increased occurrence of post-transfusion infections or cancer relapse. In this review, we report data from the literature and our laboratory dealing with interactions between antigen-presenting cells and phosphatidylserine-expressing cell dusts, including apoptotic leukocytes and blood cell-derived microparticles. Then, we discuss how these phosphatidylserine-expressing cell by-products may influence transfusion. PMID:22677430
Saas, P; Angelot, F; Bardiaux, L; Seilles, E; Garnache-Ottou, F; Perruche, S
Transfusion therapy after cardiac surgery is empirically guided, partly due to a lack of specific point-of-care hemo- stasis monitors. In a randomized, blinded, prospective trial, we studied cardiac surgical patients at moderate to high risk of transfusion. Patients were randomly assigned to either a thromboelastography (TEG)-guided transfu- sion algorithm (n 5 53) or routine transfusion therapy (n 5 52) for
Linda Shore-Lesserson; Heather E. Manspeizer; Marietta DePerio; Sanjeev Francis; Frances Vela-Cantos; M. Arisan Ergin
Hemolysis can saturate the hemoglobin (Hb)/heme scavenging system, resulting in increased circulating cell-free Hb (CF-Hb) in hereditary and acquired hemolytic disease. While recent studies have suggested a central role for intravascular hemolysis and CF-Hb in the development of vascular dysfunction, this concept has stimulated considerable debate. This highlights the importance of determining the contribution of CF-Hb to vascular complications associated with hemolysis. Therefore, a novel Hb-binding peptide was synthesized and linked to a small fragment of apolipoprotein E (amino acids 141-150) to facilitate endocytic clearance. Plasma clearance of hE-Hb-b10 displayed a rapid phase t(1/2) of 16 min and slow phase t(1/2) of 10 h, trafficking primarily through the liver. Peptide hE-Hb-B10 decreased CF-Hb in mice treated with phenylhydrazine, a model of acute hemolysis. Administration of hE-Hb-B10 also attenuated CF-Hb in two models of chronic hemolysis: Berkeley sickle cell disease (SS) mice and mice with severe hereditary spherocytosis (HS). The hemolytic rate was unaltered in either chronic hemolysis model, supporting the conclusion that hE-Hb-B10 promotes CF-Hb clearance without affecting erythrocyte lysis. Interestingly, hE-Hb-B10 also decreased plasma ALT activity in SS and HS mice. Although acetylcholine-mediated facialis artery vasodilation was not improved by hE-Hb-B10 treatment, the peptide shifted vascular response in favor of NO-dependent vasodilation in SS mice. Taken together, these data demonstrate that hE-Hb-B10 decreases CF-Hb with a concomitant reduction in liver injury and changes in vascular response. Therefore, hE-Hb-B10 can be used to investigate the different roles of CF-Hb in hemolytic pathology and may have therapeutic benefit in the treatment of CF-Hb-mediated tissue damage. PMID:23125208
Hanson, Madelyn S; Xu, Hao; Flewelen, Timothy C; Holzhauer, Sandra L; Retherford, Dawn; Jones, Deron W; Frei, Anne C; Pritchard, Kirkwood A; Hillery, Cheryl A; Hogg, Neil; Wandersee, Nancy J
Acute chest syndrome is a common cause of death among patients with sickle cell disease, and an unfamiliar condition to most Australian medical practitioners. We present a case of acute chest syndrome successfully treated with inhaled nitric oxide and exchange transfusion. In the discussion we review current and future management options of acute chest syndrome. PMID:20575992
Laurie, G A
Babesia spp. are intraerythrocytic protozoan parasites of animals and humans that cause babesiosis, a zoonotic disease transmitted primarily by tick vectors. Although a variety of species or types of Babesia have been described in the literature as causing infection in humans, the rodent parasite Babesia microti has emerged as the focal point of human disease, especially in the United States. Not only has B. microti become established as a public health concern, this agent is increasingly being transmitted by blood transfusion: estimates suggest that between 70 and 100 cases of transfusion-transmitted Babesia (TTB) have occurred over the last 30 years. A recent upsurge in TTB cases attributable to B. microti, coupled with at least 12 fatalities in transfusion recipients diagnosed with babesiosis, has elevated TTB to a key policy issue in transfusion medicine. Despite clarity on a need to mitigate transmission risk, few options are currently available to prevent the transmission of B. microti by blood transfusion. Future mitigation efforts may stress serological screening of blood donors in regionalized areas of endemicity, with adjunct nucleic acid testing during the summer months, when acute infections are prevalent. However, several hurdles remain, including the absence of a licensed blood screening assay and a thorough cost-benefit analysis of proposed interventions. Despite current obstacles, continued discussion of TTB without proactive intervention is no longer a viable alternative. PMID:21233506
Leiby, David A
Summary: Babesia spp. are intraerythrocytic protozoan parasites of animals and humans that cause babesiosis, a zoonotic disease transmitted primarily by tick vectors. Although a variety of species or types of Babesia have been described in the literature as causing infection in humans, the rodent parasite Babesia microti has emerged as the focal point of human disease, especially in the United States. Not only has B. microti become established as a public health concern, this agent is increasingly being transmitted by blood transfusion: estimates suggest that between 70 and 100 cases of transfusion-transmitted Babesia (TTB) have occurred over the last 30 years. A recent upsurge in TTB cases attributable to B. microti, coupled with at least 12 fatalities in transfusion recipients diagnosed with babesiosis, has elevated TTB to a key policy issue in transfusion medicine. Despite clarity on a need to mitigate transmission risk, few options are currently available to prevent the transmission of B. microti by blood transfusion. Future mitigation efforts may stress serological screening of blood donors in regionalized areas of endemicity, with adjunct nucleic acid testing during the summer months, when acute infections are prevalent. However, several hurdles remain, including the absence of a licensed blood screening assay and a thorough cost-benefit analysis of proposed interventions. Despite current obstacles, continued discussion of TTB without proactive intervention is no longer a viable alternative.
Leiby, David A.
Perioperative anaemia, with iron deficiency being its leading cause, is a frequent condition among surgical patients, and has been linked to increased postoperative morbidity and mortality, and decreased quality of life. Postoperative anaemia is even more frequent and is mainly caused by perioperative blood loss, aggravated by inflammation-induced blunting of erythropoiesis. Allogenic transfusion is commonly used for treating acute perioperative anaemia, but it also increases the rate of morbidity and mortality in surgical and critically ill patients. Thus, overall concerns about adverse effects of both preoperative anaemia and allogeneic transfusion have prompted the review of transfusion practice and the search for safer and more biologically rational treatment options. In this paper, the role of intravenous iron therapy (mostly with iron sucrose and ferric carboxymaltose), as a safe and efficacious tool for treating anaemia and reducing transfusion requirements in surgical patients, as well as in other medical areas, has been reviewed. From the analysis of published data and despite the lack of high quality evidence in some areas, it seems fair to conclude that perioperative intravenous iron administration, with or without erythropoiesis stimulating agents, is safe, results in lower transfusion requirements and hastens recovery from postoperative anaemia. In addition, some studies have reported decreased rates of postoperative infection and mortality, and shorter length of hospital stay in surgical patients receiving intravenous iron. PMID:23588429
Muñoz, M; Gómez-Ramírez, S; Martín-Montañez, E; Pavía, J; Cuenca, J; García-Erce, J A
The fate of 80 infants delivered after induction of labour in 72 Rh-sensitized mothers was studied to determine whether the stillbirth rate could be reduced. Labour was induced at 32 to 39 weeks of gestation; the criteria for induction were based on the history of previously affected infants, and a maternal Rh-antibody titre of 1/40 or greater, using an indirect antiglobulin technique. Nine mothers were delivered by Cesarean section. It was estimated that 26 infants were so severely affected as to be unlikely to have survived to term. However, only seven died, and one was stillborn. Two of these would normally have survived, one being Rh-negative. These two cases demonstrated the main danger in this method of management. There was a probable saving of 18 infants. In 22 mothers there was no history of previous delivery of an affected infant; in all 22 the infants survived, though six probably would not have survived to term. In 15 pregnancies in which the mothers had had a previous stillbirth, 12 infants survived. Sixty-seven infants required a total of 116 exchange transfusions. Despite the hazards it is concluded that early induction has an important place in management of Rh hemolytic disease.
Goluboff, Nathan; McKenzie, J. W. A.; Brown, Albert B.
Summary The case of a 12-year-old with a hybrid CFH/CFHL1 gene and atypical hemolytic uremic syndrome (aHUS) that had previously developed native kidney and then renal allograft loss is reported. This case illustrates the relatively common occurrence of renal loss from the late presentation of aHUS. Also presented is a protocol for the pre-emptive use of eculizumab and plasmapheresis as part of a renal transplant plan for the treatment of aHUS in patients deemed at high risk for recurrent disease. This protocol was a result of a multidisciplinary approach including adult and pediatric nephrology, transplant surgery, transfusion medicine, and infectious disease specialists. This protocol and the justifications and components of it can function as a guideline for the treatment of a group of children that have waited in limbo for the first U.S. transplant to open the door to this type of definitive care for this devastating disease.
Stewart, Zoe; Myers, David; Jetton, Jennifer; Nair, Ramesh; Reed, Alan; Thomas, Christie; Smith, Richard; Brophy, Patrick
\\u000a Since 1983 when it became apparent that the human immunodeficiency virus (HIV) could be transmitted through blood transfusions,\\u000a the number of blood transfusions administered in the United States has decreased significantly [1–7]. Before the potential\\u000a risk of HIV infection from donor blood became known, a haematocrit value of 30% and a haemoglobin concentration of 10 g\\/dl\\u000a served as the clinical
C. R. Valeri; P. Crowley; J. Loscalzo
It is now clearly established that the transfusion of blood from variant CJD (v-CJD) infected individuals can transmit the disease. Since the number of asymptomatic infected donors remains unresolved, inter-individual v-CJD transmission through blood and blood derived products is a major public health concern. Current risk assessments for transmission of v-CJD by blood and blood derived products by transfusion rely
Olivier Andréoletti; Claire Litaise; Hugh Simmons; Fabien Corbière; Séverine Lugan; Pierrette Costes; François Schelcher; Didier Vilette; Jacques Grassi; Caroline Lacroux
Homologous blood transfusion without risk is an unobtainable goal. Infection with human immunodeficiency virus continues to occur at an average rate of one infection per 100,000 transfusions, in spite of the most sensitive and specific testing available. In the past 30 years, the number of red cell antigens identified have increased from primarily ABO and Rh to some 400 antigens, which has also contributed to the hazards of blood transfusion. These risks can be minimized by the judicious use of homologous blood in conjunction with technological advances in transfusion medicine therapy and changes in attitudes of transfusionists. In the operating theater, there has been a resurgence in intraoperative autologous transfusion therapy, and patients are individualized rather than held to an arbitrary hemoglobin standard prior to anesthesia. In the preoperative period, elective surgical candidates may predeposit autologous blood or select directed donors. The prospective recipient or the directed donor may be candidate for recombinant erythropoietin therapy as a prelude to blood donation. This article discusses the uses of blood and blood products, the hazards of blood transfusion, and precautions that can be taken to minimize risks to the patient.
Posey, D. H.
Background: Hemorrhage coupled with coagulopathy remains the leading cause of preventable in-hospital deaths among trauma patients. Use of a transfusion protocol with a predefined ratio of 1:1:1 (1 each of red blood cells [RBC], frozen plasma [FP] and platelets) has been associated with improved survival in retrospective studies in military and civilian settings, but such a protocol has its challenges and may increase the risk of respiratory complications. We conducted a randomized controlled trial to assess the feasibility of a 1:1:1 transfusion protocol and its effect on mortality and complications among patients with severe trauma. Methods: We included 78 patients seen in a tertiary trauma centre between July 2009 and October 2011 who had hypotension and bleeding and were expected to need massive transfusion (? 10 RBC units in 24 h). We randomly assigned them to either the fixed-ratio (1:1:1) transfusion protocol (n = 40) or to a laboratory-results–guided transfusion protocol (control; n = 38). The primary outcome, feasibility, was assessed in terms of blood product ratios and plasma wastage. Safety was measured based on 28-day mortality and survival free of acute respiratory distress syndrome. Results: Overall, a transfusion ratio of 1:1:1 was achieved in 57% (21/37) of patients in the fixed-ratio group, as compared with 6% (2/32) in the control group. A ratio of 1:1 (RBC:FP) was achieved in 73% (27/37) in the fixed-ratio group and 22% (7/32) in the control group. Plasma wastage was higher with the intervention protocol (22% [86/390] of FP units v. 10% [30/289] in the control group). The 28-day mortality and number of days free of acute respiratory distress syndrome were statistically similar between the groups. Interpretation: The fixed-ratio transfusion protocol was feasible in our study, but it was associated with increased plasma wastage. Larger randomized trials are needed to evaluate the efficacy of such a protocol in trauma care. Trial registration: ClinicalTrials.gov, no. NCT00945542
Nascimento, Bartolomeu; Callum, Jeannie; Tien, Homer; Rubenfeld, Gordon; Pinto, Ruxandra; Lin, Yulia; Rizoli, Sandro
Hemolytic-uremic syndrome (HUS) is a rare life-threatening disorder characterized by microangiopathic hemolytic anemia, thrombocytopenia, and impaired renal function. A thrombotic microangiopathy underlies the clinical features of HUS. In the majority of cases, HUS follows an infection with toxin-producing bacteria such as verotoxin-producing Escherichia coli. In some cases, HUS is not preceded by a clinically apparent infection, and therefore, is named atypical HUS. The prognosis of atypical HUS is poor. While mortality approaches 25% during the acute phase, end-stage renal disease develops in nearly half of patients within a year. Evidence is accumulating that complement activation through the alternative pathway is at the heart of the pathophysiology leading to atypical HUS. Genetic abnormalities involving complement regulatory proteins and complement components form the molecular basis for complement activation. Since microvascular thrombosis is a quintessential feature of atypical HUS, complements and the coagulation system must work in tandem to give rise to the pathologic alterations observed in this condition. Here, a brief discussion of clinical and morphologic features of atypical HUS is followed by a concise presentation of the complement and coagulation systems. The interplay between complements and the coagulation system is graphically highlighted. Last but not least, conventional and emerging therapies for atypical HUS are outlined. PMID:24072143
Nayer, Ali; Asif, Arif
Giant cell hepatitis (GCH) with autoimmune hemolytic anemia is a rare entity, limited to young children, with an unknown pathogenesis. We report the case of 9-mo old who presented with fever, diarrhea and jaundice four days before hospitalization. Physical examination found pallor, jaundice and hepatosplenomegaly. The laboratory workup showed serum total bilirubin at 101 ?mol/L, conjugated bilirubin at 84 ?mol/L, hemolytic anemia, thrombocytopenia and immunoglobulin G (IgG) and anti-C3d positive direct Coombs’ test. The antinuclear, anti-smooth muscle and liver kidney microsomes 1 non-organ specific autoantibodies, antiendomisium antibodies were negative. Serological assays for viral hepatitis B and C, cytomegalovirus, herpes simplex and Epstein Barr virus were negative. The association of acute liver failure, Evan’s syndrome, positive direct Coomb’s test of mixed type (IgG and C3) and the absence of organ and non-organ specific autoantibodies suggested the diagnosis of GCH. The diagnosis was confirmed by a needle liver biopsy. The patient was treated by corticosteroids, immunomodulatory therapy and azathioprine but died with septicemia.
Bouguila, Jihene; Mabrouk, Sameh; Tilouche, Samia; Bakir, Dajla; Trabelsi, Amel; Hmila, Amel; Boughammoura, Lamia
Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy (TMA) which encompasses hemolytic anemia, thrombocytopenia, and organ impairment. Around 10% of cases are atypical HUS (aHUS), a rare disease with poor outcomes caused by uncontrolled activation of the alternative complement pathway. This case describes a young woman with clinical manifestations compatible with TMA during childhood and adolescence who was formally diagnosed with aHUS at the age of 21. She was managed with intensive plasma exchange and hemodialysis, which failed to improve her severe acute kidney injury and other hematological manifestations of aHUS. This was further compounded by several episodes of flash pulmonary edema and the posterior reversible encephalopathy syndrome (PRES). Treatment with the monoclonal anti-C5 inhibitor, eculizumab, improved all hematological parameters with almost full renal recovery following 3.5 months of dialysis. So far, long-term use of eculizumab (> 11 months) continues to be effective and without complication. Our case illustrates the difficulty but importance of early consideration of aHUS in patients presenting with TMA. More importantly, we highlight that near-normal renal recovery may be attained with eculizumab in adults even after a long dependence on dialysis - an observation that has not been reported in the literature so far. PMID:23557793
Povey, Hannah; Vundru, Rahul; Junglee, Naushad; Jibani, Mahdi
Genetic mutations in complement components are associated with the development of atypical hemolytic uremic syndrome (aHUS), a rare disease with high morbidity rate triggered by infections or unidentified factors. The uncontrolled activation of the alternative pathway of complement results in systemic endothelial damage leading to progressive development of renal failure. A previously healthy 8-month-old boy was referred to our hospital because of onset of fever, vomiting, and a single episode of nonbloody diarrhea. Acute kidney injury with preserved diuresis, hemolytic anemia, and thrombocytopenia were detected, and common protocols for management of HUS were followed without considerable improvement. The persistent low levels of complement component C3 led us to hypothesize the occurrence of aHUS. In fact, the child carried a specific mutation in complement factor H (Cfh; nonsense mutation in 3514G>T, serum levels of Cfh 138 mg/L, normal range 350-750). Given the lack of response to therapy and the occurrence of kidney failure requiring dialysis, we used eculizumab as rescue therapy, a monoclonal humanized antibody against the complement component C5. One week from the first administration, we observed a significant improvement of all clinical and laboratory parameters with complete recovery from hemodialysis, even in the presence of systemic infections. Our case report shows that complement inhibiting treatment allows the preservation of renal function and avoids disease relapses during systemic infections. PMID:23027168
Giordano, Mario; Castellano, Giuseppe; Messina, Giovanni; Divella, Claretta; Bellantuono, Rosa; Puteo, Flora; Colella, Vincenzo; Depalo, Tommaso; Gesualdo, Loreto
A 74-year-old man was admitted to hospital because of persistent fever, diarrhea, and abdominal pain. CT scanning showed extensive wall thickening of the colon. He was transferred to our hospital because he further developed ascites and paraaortic lymph node swelling. On presentation, he was extremely emaciated with superficial lymph node swelling, ascitic signs, and leg edema. Histological image of a biopsied mesenteric lymph node demonstrated diffuse infiltration of large abnormal T cells. Surface antigen analysis of abnormal cells in the ascites revealed positivity for CD3, CD8, CD56, and weak positivity for CD103. Polymerase chain reaction analysis showed monoclonal rearrangement of the T cell receptor (TCR) gene. The subtype of TCR was ??. A diagnosis of enteropathy-associated T cell lymphoma (EATL) type II was made. The lymphoma involved the bone marrow. The patient also had severe hemolytic anemia with a positive Coomb's test result. An additional diagnosis for autoimmune hemolytic anemia (AIHA) was made, which was resistant to methylprednisolone therapy. We first treated him with only vincristine in addition to the steroid to avoid acute tumor lysis syndrome ; however, he died of septic shock that occurred soon after vincristine administration. To the best of our knowledge, this may be the first reported case of EATL complicated by AIHA. PMID:22104311
Kato, Aiko; Takiuchi, Yoko; Aoki, Kazunari; Ono, Yuichiro; Arima, Hiroshi; Nagano, Seiji; Tabata, Sumie; Yanagita, Soshi; Matsushita, Akiko; Maruoka, Hayato; Wada, Masaya; Imai, Yukihiro; Ishikawa, Takayuki; Takahashi, Takayuki
Direct antiglobulin test (DAT) with only complement detected on red blood cells is a rare laboratory finding, and its significance in the setting of warm autoimmune hemolytic anemia (AIHA) is controversial. During 2 years (2003-2004) 277 patients with positive DAT were recorded in the blood bank registries, 17 of them had DAT reactive with C3 alone with no cold agglutinin or other nonimmune causes for hemolysis diagnosed. Red cell eluate disclosed small amounts of IgG in two patients. In nine patients no signs of clinical hemolysis were found, however, all these patients had underlying conditions that are known to be associated with red cells autoantibodies (autoimmune disorder or malignancy). Eight patients developed AIHA, seven of them with severe hemolysis. Three patients had idiopathic AIHA, and the others have been diagnosed with infectious, lymphoproliferative and autoimmune disorders. In two patients with acute infection the hemolytic process spontaneously resolved, three responded to corticosteroid therapy, while three patients were refractory to two lines of drug therapy and underwent splenectomy. Reticulocytopenia was found in four patients. Our results emphasize that AIHA with DAT reactive with complement alone is a rare disorder and might be accompanied by severe, refractory to conventional treatment and life-threatening hemolysis. PMID:18983301
Shvidel, L; Shtalrid, M; Duek, A; Haran, M; Berrebi, A; Sigler, E
Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. Mutations in genes encoding regulators of the alternative complement pathway (CFH, MCP, C3, CFI, CFB, THBD, and CFHR1-5) are connected with this disease. Polymorphisms (SNPs) in these genes might also influence the manifestation of aHUS. We have analyzed the genes of CFH, CFI, MCP, and C3 in a cohort of 10 unrelated Czech patients with clinically diagnosed familial aHUS. Surprisingly, 4 patients had mutations only in MCP, without mutations in any of the other genes that cause aHUS. Mutations, as yet unpublished, were widely distributed over the gene (SCR2 domain, signal peptide, and cytoplasmic region). The phenotype of the patients and their close relatives (14 individuals) was also investigated. Functional examination of MCP was also provided and proved lower expression on granulocytes in all mutations. Severity of disease varied, but onset was never earlier than 5 years of age. Penetrance of disease was 50% among carriers. We found that the severity and recurrence of the disease within families varied and might also be dependent on SNPs. Mutations in the MCP gene seems to be a common etiology of aHUS in Czech patients. PMID:21706448
Provaznikova, Dana; Rittich, Simon; Malina, Michal; Seeman, Tomas; Marinov, Iuri; Riedl, Magdalena; Hrachovinova, Ingrid
OBJECTIVE—To determine the incidence, complications, management, and outcome in infants with twin-twin transfusion syndrome (TTTS) over a period of five years.?METHODS—TTTS was diagnosed in monochorionic twins if one was pale and the other plethoric with a haemoglobin difference ? 5 g/100 ml and/or birthweight differences ? 15%.?RESULTS—Eighteen (6.2%) of the 292 twin pairs had TTTS. Eight pairs (44%) had the acute type and the rest (56%) had the chronic type of TTTS. The mean (SEM) intrapair haemoglobin difference in the acute type was 4.8 (2.1) g/100 ml which gave a discordance of 7.1 (4.6)%, whereas that in the chronic type was 6.9 (2.9) g/100 ml and 24.4 (6.1)% respectively. Infants with the acute type had a significantly higher incidence of vaginal delivery (p < 0.03), hypotension (p < 0.025), and respiratory distress (p < 0.01) compared with those with the chronic type. There was no significant difference in the incidence of anaemia, polycythaemia, asphyxia, hypoglycaemia, and hyperbilirubinaemia. Two recipients died in utero as the result of chronic TTTS, while their survivors developed spastic cerebral palsy. There were no neonatal deaths.?CONCLUSIONS—TTTS, although uncommon, may have an adverse neurodevelopmental outcome especially if one twin dies in utero. Prompt recognition and management of the haemodynamic and haematological problems of infants with the acute types of TTTS will result in optimal neurodevelopmental outcome.??
Seng, Y; Rajadurai, V
DEAP-HUS [Deficiency of CFHR (complement factor H-related) plasma proteins and Autoantibody Positive form of Hemolytic Uremic Syndrome] represents a novel subtype of hemolytic uremic syndrome (HUS) with unique characteristics. It affects children and requires special clinical attention in terms of diagnosis and therapy. DEAP-HUS and other atypical forms of HUS share common features, such as microangiopathic hemolytic anemia, acute renal failure, and thrombocytopenia. However, DEAP-HUS has the unique combination of an acquired factor in the form of autoantibodies to the complement inhibitor Factor H and a genetic factor which, in most cases, is the chromosomal deletion of a 84-kbp fragment within human chromosome 1 that results in the absence of the CFHR1 and CFHR3 proteins in plasma. Special attention is required to diagnose and treat DEAP-HUS patients. Most patients show a favorable response to the reduction of autoantibody titers by either plasma therapy, steroid treatment, and/or immunosuppression. In addition, in those DEAP-HUS patients with end-stage renal disease, the reduction of autoantibody titers prior to transplantation is expected to prevent post-transplant disease recurrence by aiming for full complement control at the endothelial cell surface in order to minimize adverse complement and immune reactions. PMID:20157737
Zipfel, Peter F; Mache, Christoph; Müller, Dominik; Licht, Christoph; Wigger, Marianne; Skerka, Christine
Two cationic proteins, C1 and C3, were purified to homogeneity from the hemolytic fraction of the venom of Bunodosoma caissarum sea anemone. The purification processes employed gel filtration followed by ion exchange chromatography, being the purity and molecular mass confirmed by SDS-PAGE and mass spectrometry. Protein C1 represented the second major peak of the hemolytic fraction and was previously believed to be a cytolysin belonging to a new class of hemolysins. The C1 protein has a molecular mass of 15495 Da and was assayed for hemolysis, PLA2 activity and acute toxicity in crabs and mice, showing no activity in these assays. It has an amino terminal with no similarity to all known hemolysins and, therefore, should not be considered a toxin, being its function completely unknown. The protein C3 (19757 Da), that also lacks PLA2 activity, was recognized by antiserum against Eqt II and presented high hemolytic activity to human erythrocytes (ED50 of 0.270 microg/ml), being named Caissarolysin I (Bcs I). Its activity was inhibited by pre-incubation with sphingomyelin (SM) and also when in presence of erythrocytes pre-treated with the SMase P2, a phospholipase D from the brown spider Loxosceles intermedia, indicating that SM is the main target of Bcs I. Caissarolysin I is the first hemolysin purified from a sea anemone belonging to the genus Bunodosoma and belongs to the Actinoporin family of sea anemone hemolysins. PMID:16458433
de Oliveira, Joacir Stolarz; Zaharenko, André Junqueira; de Freitas, José Carlos; Konno, Katsuhiro; de Andrade, Sonia A; Portaro, Fernanda C V; Richardson, Michael; Sant'anna, Osvaldo Augusto; Tambourgi, Denise V
normalization of the patient's vital signs. This includes achieving a mean arterial pressure greater than 70 mmHg and systolic blood pressure > 90 mmHg. Patients with blood loss may require blood transfusion. Current transfusion triggers adopted in veterinary medicine for the acutely anemic or critically ill dog, are a PCV of 24-27%, and in the cat a PCV of 15-20%.
Blood transfusions are associated with recurrence of solid cancers. Angiogenesis is essential for cancer growth. Our aim was to determine for the first time in a prospective cohort study the effect of prestorage allogeneic leucodepleted SAGM (saline, adenine, glucose, mannitol) red cell transfusion on angiogenic factor levels and in vitro angiogenesis. Forty pretransfusion adult hospital inpatients were selected consecutively. Serum vascular endothelial growth factor (VEGF) and endostatin were measured in each patient before and after prestorage allogeneic leucodepleted SAGM red cell transfusion. All samples were exposed to an in vitro endothelial cell proliferation assay and 10 sample groups were also exposed to an in vitro whole angiogenesis assay. The median number of units transfused was 2 (minimum-maximum, 2-4). Twenty-nine (73%) patients had a rise in VEGF, with an overall increase of 118 pg/ml (quartiles -5, 306; P < 0.01). Twenty-eight (70%) patients had a decrease in endostatin, with an overall reduction of 1.2 ng/ml (quartiles 4.0, 0.0; P = 0.017). There was an overall 33% increase in endothelial cell proliferation (P < 0.01) and a 9.4% increase in in vitro whole assay angiogenesis (P < 0.01). Prestorage allogeneic leucodepleted SAGM red cell transfusions are associated with a favourable angiogenic factor imbalance and an elevation in in vitro angiogenesis. PMID:15053807
Patel, H B; Nasir, F A; Nash, G F; Scully, M F; Kakkar, A K
cases of mild to moderate anemia are still debated.3 A large body of literature documents the many potential harms asso- ciated with blood transfusions.4 In this review, we explore the rationale and summarize the evidence for blood conservation strategies to reduce acute blood loss and prevent subacute anemia in critically ill patients (Table 1 and Table 2). (A de- scription
Alan T. Tinmouth; Lauralynn A. McIntyre; Robert A. Fowler
Splenic infarction is most commonly caused by cardiovascular thromboembolism; however, splenic infarction can also occur in hematologic diseases, including sickle cell disease, hereditary spherocytosis, chronic myeloproliferative disease, leukemia, and lymphoma. Although 10% of splenic infarction is caused by hematologic diseases, it seldom accompanies autoimmune hemolytic anemia (AIHA). We report a case of a 47-year-old woman with iron deficiency anemia who presented with pain in the left upper abdominal quadrant, and was diagnosed with AIHA and splenic infarction. Protein C activity and antigen decreased to 44.0% (60-140%) and 42.0% (65-140%), respectively. Laboratory testing confirmed no clinical cause for protein C deficiency, such as disseminated intravascular coagulation, sepsis, hepatic dysfunction, or acute respiratory distress syndrome. Protein C deficiency with splenic infarction has been reported in patients with viral infection, hereditary spherocytosis, and leukemia. This is a rare case of splenic infarction and transient protein C deficiency in a patient with AIHA. PMID:22259634
Park, Min Yong; Kim, Jung A; Yi, Seong Yoon; Chang, Sun Hee; Um, Tae Hyun; Lee, Hye Ran
The route of infection in acute suppurative thyroiditis is unknown in most cases; when demonstrated, pyriform sinus fistula appears to be the most frequent one. We report the clinical and laboratory findings of a child in whom culture of the thyroid pus yielded two bacteria which are part of the normal oropharyngeal flora: capnocytophaga ochracea and group F Beta-hemolytic streptococcus. The preliminary results of the culture, which showed a mixed flora, prompted us to search and to find a pyriform sinus fistula. Apart from the onset in infancy, the left lobe involvement and the frequent recurrence, the recovery from the thyroid pus of bacteria from normal oropharyngeal flora should be included in the characteristic features of thyroiditis resulting from an infection through the pyriform sinus fistula. PMID:3959182
Goudreau, E; Comtois, R; Bayardelle, P; Beauregard, H; Larochelle, D
The twin-twin transfusion syndrome, associated with acute polyhydramnios in the 18th to 28th week of gestation, has a high perinatal mortality rate. Patients managed without intervention have essentially a 100% mortality rate for the involved twins. Different methods of intervention have been described, including therapeutic amniocentesis, selective feticide, and placental vessel puncture. In this case report we describe selective removal
Michael A. Urig; Gary F. Simpson; John P. Elliott; William H. Clewell
Three patients with maple syrup urine disease were treated during the acute neonatal stage. Multiple exchange transfusions proved to be a satisfactory means of achieving rapid clinical and biochemical improvement during this phase. On the other hand, evidence is provided suggesting that in addition to exchange transfusions, a high calorie intake above 150 Cal\\/kg body weight\\/day is necessary to lower
G. Hammersen; L. Wille; H. Schmidt; P. Lutz; H. Bickel
Porphyrias, a group of inborn errors of heme synthesis, are classified as hepatic or erythropoietic according to clinical data and the main site of expression of the specific enzymatic defect. Hereditary coproporphyria (HC) is an acute hepatic porphyria with autosomal dominant inheritance caused by deficient activity of coproporphyrinogen III oxidase (COX). Typical clinical manifestations of the disease are acute attacks of neurological dysfunction; skin photosensitivity may also be present. We report a variant form of HC characterized by a unifying syndrome in which hematologic disorders predominate: harderoporphyria. Harderoporphyric patients exhibit jaundice, severe chronic hemolytic anemia of early onset associated with hepatosplenomegaly, and skin photosensitivity. Neither abdominal pain nor neuropsychiatric symptoms are observed. COX activity is markedly decreased. In a first harderoporphyric family, with three affected siblings, a homozygous K404E mutation has been previously characterized. In the present study, molecular investigations in a second family with neonatal hemolytic anemia and harderoporphyria revealed two heterozygous point mutations in the COX gene. One allele bore the missense mutation K404E previously described. The second allele bore an A-->G transition at the third position of the donor splice site in intron 6. This new COX gene mutation resulted in exon 6 skipping and the absence of functional protein production. In contrast with other COX gene defects that produce the classical hepatic porphyria presentation, our data suggest that the K404E substitution (either in the homozygous or compound heterozygous state associated with a mutation leading to the absence of functional mRNA or protein) is responsible for the specific hematologic clinical manifestations of harderoporphyria. PMID:9454777
Lamoril, J; Puy, H; Gouya, L; Rosipal, R; Da Silva, V; Grandchamp, B; Foint, T; Bader-Meunier, B; Dommergues, J P; Deybach, J C; Nordmann, Y
This study was aimed to observe and analyze the effectiveness of platelet transfusion. The platelet count of 1786 patients before transfusion and on 20-24 hours after transfusion was determined by using Auto-Hematology Analyzer, the percent platelet recovery (PPR) was calculated, the platelet transfusion efficiency (PTE) was evaluated by PPR and hemorrhage presentation after platelet transfusion, and the PTE was statistically analyzed according to disease cause, transfusion frequency, platelet type and once transfusion amount. The results showed that the total PTE of 1786 patients was 52.5%. The comparion of PTE among groups of diesease cause showed that PTE in luekemia and aplastic anemia(AA) was lowest, as compared with that of other diseases (P < 0.05), while PTE in operation group was highest. The comparison of PTE among groups of transfusion frequency revealed also statistical difference (P < 0.01), meanwhile PTE decreased with increasing of transfusion frequency. The comparion of PTE among groups of platelet type (platelet phoresis or platelet concentrate) showed statistical difference (P < 0.01). The comparison of PTE among groups of platelet concentrate of once transfusion amount showed no stastistcal difference (P > 0.05). It is concluded that the PTE closely relates with disease cause of patients, moreover transfusion frequency also associates with PTE, the more frequency of transfusion, the higher possibility of transfusion refractoriness. The PTE of platelet pheresis is obviously superior to that of platelet concentrate, while PTE of platelet concentrate not significantly relates with once adeguate or not. PMID:23998608
Yang, Mei; Luo, Hong; Shu, Bin; An, Bang-Quan; Xia, Shi-Qin; Wang, Mao-Ling
Haemophilus ducreyi is the causative agent of the sexually transmitted disease chancroid. We have identified a hemolytic activity expressed by H. ducreyi. This activity is most readily detected when horse erythrocytes are used as a target; however, low levels of activity can be detected with sheep, human, or rabbit erythrocyte targets. The activity is heat labile and protease sensitive.
Palmer, K L; Grass, S; Munson, R S
Background and objectives: Atypical hemolytic uremic syndrome (aHUS) is associated with a congenital or acquired dysregulation of the complement alternative pathway that leads to continuous complement activation on host cells causing inflammation and damage. Eculizumab, a humanized mAb against complement protein C5, inhibits activation of the terminal complement pathway. Design, setting, participants, & measurements: We report an adolescent with relapsing unclassified aHUS. On admission, a high plasma creatinine level indicated a poor prognosis, and hemodialysis had to be started. Plasma exchanges were initially effective against the microangiopathic hemolytic activity and allowed a temporary improvement of renal function with termination of hemodialysis after 7 wk. Subsequently, plasma exchanges (three times per week) failed to prevent ongoing aHUS activity and progressive renal failure. After 12 wk, aHUS treatment was switched to eculizumab. Results: Eculizumab was effective in terminating the microangiopathic hemolytic process in two aHUS relapses; however, after normalization of complement activity, aHUS recurred and ultimately led to anuric end-stage renal failure. Conclusions: In this patient, complement inhibition by eculizumab temporarily terminated the microangiopathic hemolytic activity. Nevertheless, renal damage as a result of preceding and subsequent aHUS activity resulted in end-stage renal failure; therefore, therapeutic success may depend on early administration of eculizumab. The optimal duration of treatment may be variable and remains to be determined.
Acham-Roschitz, Birgit; Fremeaux-Bacchi, Veronique; Kirschfink, Michael; Zipfel, Peter F.; Roedl, Siegfried; Vester, Udo; Ring, Ekkehard
Chronic lymphocytic leukemia (CLL) is a malignant clonal expansion of CD5+B lymphocytes. The CD5+B lymphocytes have been postulated to produce autoantibodies. CLL patients may demonstrate features of autoimmunity including autoimmune hemolytic anemia. However, the origin of the autoantibodies causing the hemolysis is not clear. The present studies were performed to determine whether these autoantibodies are the products of the neoplastic B-CLL clones. Immunoglobulins (Ig) were eluted from washed red blood cells (RBC) obtained from two CLL patients at the time they had autoimmune (DAT-direct antiglobulin test-positive) hemolytic anemia. The light chain phenotypes of these eluted autoantibodies were determined and found to be monotypic with exact correlation to the light chain expressed on the surface of the B-CLL clones. Elutions from RBC of DAT negative patients or normal volunteers failed to demonstrate measurable amounts of Ig. In contrast, Ig eluted from RBC obtained from SLE patients with DAT positive hemolytic anemia found to be polyclonal autoantibodies exhibiting both light chain types. Furthermore, CD5+B lymphocytes obtained from the same two CLL patients (DAT+) produce, in vitro understimulation with phorbal myristate acetate (PMA), monoclonal antibodies which react and bind to RBC. Thus these studies provide direct evidence demonstrating that the antibodies causing the autoimmune hemolytic anemia in our two CLL patients are the products of the B-CLL neoplastic clones. PMID:7690517
Sthoeger, Z M; Sthoeger, D; Shtalrid, M; Sigler, E; Geltner, D; Berrebi, A
We conducted prospective surveillance of childhood hemolytic uremic syndrome (HUS) from 1997 to 2001 to describe disease incidence and clinical, epidemiologic and microbiologic characteristics. We compared our findings, where possible, with those of a previous study conducted from 1985 to 1988. The average annual incidence of HUS for the United Kingdom and Ireland (0.71\\/100,000) was unchanged from 1985 to 1988.
Richard M. Lynn; Sarah J. O'Brien; C. Mark Taylor; Goutam K. Adak; Henrik Chart; Tom Cheasty; John E. Coia; Iain A. Gillespie; Mary E. Locking; William J. Reilly; Henry R. Smith; Aoife Waters; Geraldine A. Willshaw
Green staining of the dentition is a phenomenon associated with the deposition of bilirubin in the matrix of hard tissue during formation. This article presents a case of green teeth in a patient born 28 weeks premature with a medical history of hemolytic jaundice and grade IV intraventricular hemorrhage at birth. PMID:23823340
Rammal, M; Meador, M; Rodriguez, M; Lish, B
This paper provides a brief history of the evolution of the Jehovah's Witness faith with a short discussion on the biblical justification for followers' refusal of blood transfusions. It also briefly considers the ethical principles leading to potential conflicts between health care workers and Jehovah's Witnesses patients and examines several significant legal rulings in the United States and Canada that caregivers should be aware of. A discussion of what blood products are and are not currently acceptable is also presented. Finally, the impact of the Jehovah's Witness reform movement aimed at allowing blood transfusions and the nature of recent doctrinal shifts in the Jehovah's Witness faith on the matter of blood transfusions are discussed. PMID:12237734
Doyle, D John
Transfusion medicine has become a large and complex specialty. Although there are now systematic reviews covering many aspects of transfusion, these span a large number of clinical areas and are published across more than a hundred different medical journals, making it difficult for transfusion medicine practitioners and researchers to keep abreast of the current high-level evidence. In response to this problem, NHS Blood and Transplant's Systematic Review Initiative (SRI) has produced a comprehensive overview of systematic reviews in transfusion medicine. A systematic search (to December 2009) and screening procedure were followed by the appraisal of systematic reviews according to predefined inclusion criteria. The 340 eligible systematic reviews were mapped to 10 transfusion intervention groups and 14 topic groups within clinical medicine. Trends in the systematic review literature were examined and gaps in the literature described. The spread of systematic reviews across clinical areas was found to be very uneven, with some areas underreviewed and others with multiple systematic reviews on the same topic, making the identification of the best evidence for current transfusion practice a continuing challenge. References and links to all systematic reviews included in this overview can be freely accessed via the SRI's new online database, the Transfusion Evidence Library (www.transfusionguidelines.org). PMID:20851331
Dorée, Carolyn; Stanworth, Simon; Brunskill, Susan J; Hopewell, Sally; Hyde, Chris J; Murphy, Mike F
Babesiosis, a common disease of animals, can infect humans via vector “tick bite”, particularly in endemic areas. The recent reports of fatal cases in Hepatitis C and postliver transplant patients resulting from transfusion of contaminated blood should alert the medical profession regarding this emerging dilemma in endemic as well as nonendemic areas and the need for accurate blood screening for transfusion. Here, we illustrate different stages of the parasite lifecycle, progression of babesiosis in animal model, some aspects of pathologic outcomes, ongoing therapeutic modalities, and a feasible Acridine Orange fluorescent methodology for the diagnostic evaluation of blood samples.
Oz, Helieh S.; Westlund, Karin H.
H. ducreyi is the causative agent of chancroid, a genital ulcer disease most prevalent in developing countries. Chancroid enhances the heterosexual transmission of human immunodeficiency virus and is identified in focal outbreaks in the United States, but little is known about its pathogenesis. We studied the hemolysin produced by H. ducreyi because this molecule might be an important virulence factor in the pathogenesis of chancroid. Ten strains of H. ducreyi were tested on newly devised blood agar plates and were found to have hemolytic activity. We examined the hemolytic activity of H. ducreyi 35000 further and found that it was heat labile, cell associated, greatest at pH 7.0, and produced in logarithmic- but not stationary-phase cultures. Using transposons Tn916 and Tn1545-delta 3, we have isolated three classes of transposon mutants of strain 35000: those with no detectable hemolytic activity, those with reduced hemolytic activity, and those with enhanced hemolytic activity. Transposon insertions in the nonhemolytic mutants were located in a DNA sequence which hybridized to the Proteus mirabilis hemolysin gene. Analysis of clones containing overlapping sections of this region served to further localize the H. ducreyi hemolysin gene and allow its expression in Escherichia coli and complementation of the nonhemolytic defect in an H. ducreyi mutant. These experiments indicate that H. ducreyi 35000 produces a hemolysin that is related to the calcium-independent hemolysin produced by P. mirabilis. Further experiments are needed to define the similarity of the H. ducreyi hemolysin to other calcium-independent hemolysins and to determine its role in the pathogenesis of chancroid.
Totten, P A; Norn, D V; Stamm, W E
A total of 130 cordocenteses, including 96 intravascular fetal blood transfusions, were performed in 21 pregnancies complicated by red cell isoimmunization. Transfusions were commenced at 18–34 weeks’ gestation and repeated up to 7 times, at 1- to 4-week intervals. The volumes of transfused blood were 5–150 ml, the haematocrits 62–88 % and the rate of transfusions 1–15 ml\\/min. The pretransfusion
K. H. Nicolaides; P. W. Soothill; C. H. Rodeck; W. Clewell
Identification of a universal “transfusion trigger” has eluded surgeons for years. Optimization of cardiopulmonary hemodynamics should precede the decision to transfuse red blood cells. Red blood cell transfusion should be considered when global oxygen delivery falls below a critical point with increasing oxygen extraction and lactate levels. At present, these parameters must be monitored with invasive techniques. This article addresses
A. Gerson Greenburg
Acute postinfectious glomerulonephritis are defined by an acute nonsuppurative inflammatory insult predominantly glomerular. Its current incidence is uncertain because of the frequency of subclinical forms. The most common infectious agent involved is beta hemolytic streptococcus group A. Acute postinfectious glomerulonephritis is uncommon in adults, and its incidence is progressively declining in developed countries. Humoral immunity plays a key role in the pathogenesis of kidney damage. Complement activation by the alternative pathway is the dominant mechanism, but a third way (lectin pathway) has been recently identified. The classic clinical presentation is sudden onset of acute nephritic syndrome after a free interval from a streptococcal infection. Treatment is essentially symptomatic and prevention is possible through improved hygiene and early treatment of infections. PMID:22483748
Ramdani, Benyounès; Zamd, Mohamed; Hachim, Khadija; Soulami, Kenza; Ezzahidy, Madiha; Souiri, Malika; Fadili, Wafaa; Lahboub, Assia; Hanafi, Leila; Boujida, Meryem; Squalli, Saida; Benkirane, Amal; Benghanem, Mohamed Gharbi; Medkouri, Ghizlane
Attempts to detect infectivity in the blood of humans and animals affected with transmissible spongiform encephalopathies (TSEs or prion diseases) have often been inconclusive because of the limitations of cross-species bioassays and the small volumes of blood that can be injected by the intracerebral route. A model has been developed for the experimental study of TSE transmission by blood transfusion
Nora Hunter; James Foster; Angela Chong; Sandra McCutcheon; David Parnham; Samantha Eaton; Calum MacKenzie; Fiona Houston
Our interest is in enzymatically converting type A and B erythrocytes to type O under conditions which render them useful in transfusion therapy. We are currently attempting such conversions of A cells using an alpha-N-acetylgalactosamindase (A-zyme) for ...
The use of therapeutic plasma has increased in France by more than 40% since 2002. This growth may be explained by the improvement in transfusion safety, the diminution of the risk of transmission of pathogens and the regained confidence of the physicians in blood products. Therapeutic plasma also benefits from additional procedures to reduce infectious (securisation) or immunological risks (selection of blood donors). Its application in massive transfusions has undergone a significant evolution over the last few years. A proactive attitude favouring early and important use of plasma on the basis of pre-established protocols is advocated henceforth. The prescription of therapeutic plasma for other indications must be guided by the results of biological tests and an evaluation of the haemorrhagic risk. Despite regular updating of the guidelines for good transfusion practice, plasma is still sometimes prescribed for prophylactic purposes in situations where the biological and/or clinical criteria do not justify it. Moreover, it is not recommended to use fresh frozen plasma in cases of deficiency of coagulation factors if the specific concentrates are available as intravenous fluids. Complementary clinical studies will be necessary to evaluate, in certain indications, the real benefits of the transfusion of plasma and the interest of replacing it by concentrates of coagulant factors (fibrinogen, prothrombin complex). PMID:23587624
Critically ill children in pediatric intensive care units are commonly indicated for blood transfusion due to many reasons. Children are quite different from adults during growth and development, and that should be taken into consideration. It is very difficult to establish a universal transfusion guideline for critically ill children, especially preterm neonates. Treating underlying disease and targeted replacement therapy are the most effective approaches. Red blood cells are the first choice for replacement therapy in decompensated anemic patients. The critical hemoglobin concentration may be higher in critically ill children for many reasons. Whole blood is used only in the following conditions or diseases: (1) exchange transfusion; (2) after cardiopulmonary bypass; (3) extracorporeal membrane oxygenation; (4) massive transfusion, especially in multiple component deficiency. The characteristics of hemorrhagic diseases are so varied that their therapy should depend on the specific needs associated with the underlying disease. In general, platelet transfusion is not needed when a patient has platelet count greater than 10,000/mm3 and is without active bleeding, platelet functional deficiency or other risk factors such as sepsis. Patients with risk factors or age less than 4 months should be taken into special consideration, and the critical thrombocyte level will be raised. Platelet transfusion is not recommended in patients with immune-mediated thrombocytopenia or thrombocytopenia due to acceleration of platelet destruction without active bleeding or life-threatening hemorrhage. There are many kinds of plasma-derived products, and recombinant factors are commonly used for hemorrhagic patients due to coagulation factor deficiency depending on the characteristics of the diseases. The most effective way to correct disseminated intravascular coagulation (DIC) is to treat the underlying disease. Anticoagulant therapy is very important; heparin is the most common agent used for DIC but the results are usually not satisfactory. Antithrombin III, protein C, or recombinant thrombomodulin has been used successfully to treat this condition. For reducing the risk of organism transmission and adverse reactions resulting from blood transfusion, the following measures have been suggested: (1) replacement therapy using products other than blood (e.g., erythropoietin, iron preparation, granulocyte colony-stimulating factor); (2) special component replacement therapy for specific diseases; (3) autotransfusion; (4) subdividing whole packed blood products into smaller volumes to reduce donor exposure; (5) advances in virus-inactivating procedures. To avoid viral transmission, vapor-heated or pasteurized products and genetic recombinant products are recommended. Cytomegalovirus (CMV)-seronegative blood, leukoreduced and/or irradiated blood are recommended for prevention of CMV infection, graft-versus-host-disease and alloimmunization in neonate and immunocompromised patient transfusion. There is no reason to prescribe a plasma product for nutritional supplementation because of the risk of complications. The principle: complications of transfusion must be avoided, the rate of blood exposure should be reduced and the safety of the transfused agents or components should be maintained must always be kept in mind. PMID:18947009
A technique of fetal transfusion using sonographic guidance for needle placement is described. Sixteen patients underwent a total of 43 transfusions. The fetal mortality risk appears to be lower than that encountered in fluoroscopy-directed transfusions. Fetal irradiation has been dramatically reduced from an average of 2.2 rads per transfusion to 0.29 rads per transfusion. The technique allows very precise placement of a needle within the uterus or fetus. We have used it in difficult cases for amniocentesis. The technique could also be adapted to other problems requiring accurate placement of a needle within the body, such as biopsy of masses or aspiration of cysts. PMID:7243107
Clewell, W H; Dunne, M G; Johnson, M L; Bowes, W A
Anemia is common in critically ill patients. As a consequence packed red blood cell (PRBC) transfusions are frequent in the critically ill. Over the past two decades a growing body of literature has emerged, linking PRBC transfusion to infections, immunosuppression, organ dysfunction, and a higher mortality rate. However, despite growing evidence that risk of PRBC transfusion outweighs its benefit, significant numbers of critically ill patients still receive PRBC transfusion during their intensive care unit (ICU) stay. In this paper, we summarize the current literature concerning the impact of anemia on outcomes in critically ill patients and the potential complications of PRBC transfusions.
Athar, M. Kamran; Puri, Nitin; Gerber, David R.
Unique risks of red blood cell transfusions in very-low-birth-weight neonates: associations between early transfusion and intraventricular hemorrhage and between late transfusion and necrotizing enterocolitis.
Abstract Red blood cell transfusions can be life-saving for neonates with severe anemia or active hemorrhage. However, risks of transfusions exist and should always be weighed against potential benefits. At least two transfusion risks are unique to very low birth weight neonates. The first is an association between transfusions given in the first days after birth and the subsequent occurrence of a grade 3 or 4 intraventricular hemorrhage. The second is an association between "late" RBC transfusions and the subsequent occurrence of necrotizing enterocolitis. Much remains to be discovered about the pathogenesis of these two outcomes. Moreover, work is needed to clearly establish whether transfusions are causatively-associated with these outcomes or are co-variables. This review will provide basic data establishing these associations and propose mechanistic explanations. PMID:24059555
Christensen, Robert D; Baer, Vickie L; Del Vecchio, Antonio; Henry, Erick
In this study significantly prolonged canine renal allograft survival has been demonstrated after transfusion of 100 ml of third-party whole blood given peroperatively. Peroperative transfusions of third-party leukocyte-free blood or pure lymphocyte cell suspensions did not influence graft survival. Furthermore, no improvement in graft survival has been found after a peroperative transfusion of irradiated whole blood (2500 rad). These data suggest that delayed graft rejection after blood transfusions can only be expected after the administration of whole blood. The role of competent lymphocytes in whole blood is questionable, since a transfusion or irradiated whole blood in combination with nonirradiated lymphocytes did not lead to prolonged graft survival. Immunosuppression of the recipient directly after transfusion seems to be essential to induce the beneficial effect of blood transfusions. This has been demonstrated for a transfusion of whole blood 14 days before transplantation. A single transfusion of 100 ml of whole blood 14 days before transplantation could effectively prolong graft survival if immunosuppression with azathioprine and prednisone was started on the day of transfusion. No improvement in graft survival has been found with such a transfusion if preoperative immunosuppression has been omitted.
van der Linden, C.J.; Buurman, W.A.; Vegt, P.A.; Greep, J.M.; Jeekel, J.
A 26-year-old previously healthy female presented with a 6-month history of anemia. The laboratory findings revealed hemolytic anemia and direct antiglobulin test was positive. With a diagnosis of autoimmune hemolytic anemia (AIHA), prednisolone was started but was ineffective after 1 month of therapy. A bone marrow trephine biopsy revealed pure red cell aplasia (PRCA) showing severe erythroid hypoplasia. The case was considered PRCA following AIHA. This combination without clear underlying disease is rare. Human parvovirus B19 infection was not detected in the marrow aspirate during reticulocytopenia. The patient received azathioprine, and PRCA improved but significant hemolysis was once again documented with a high reticulocyte count. The short time interval between AIHA and PRCA phase suggested an increased possibility of the evolution of a single disease. PMID:23525059
Saha, M; Ray, S; Kundu, S; Chakrabarti, P
The aim of this study was the analysis of drug-resistance and frequency appearance of beta-hemolytic streptococci strains which were isolated in 2003-2005 in the University Hospital at the L. Rydygier Collegium Medicum in Bydgoszcz University of Nicolaus Copernicus in Toru?. Among investigeted beta-hemolytic streptococci the most frequency isolated species was S. agalactiae. All isolates examined in our study were susceptible to penicillin, the higest rate of resistance was found for tetracycline. The rates of resistence to macrolide-lincosamide-streptogramin B (phenotyp MLS(B)) were as follows: S. agalactiae (18.7%), S. pyogenes (10.1%), group G streptococci (10.6%) and group C streptococci (8.0%). In our study we presented also a special case patient from which in investigeted period S. agalactiae was isolated twenty eight times. For ten chromosomal DNA isolated from this patient three different PFGE profiles were obtained. PMID:18416122
Miko?ajczyk, Dorota; Budzy?ska, Anna; Kaczmarek, Agnieszka; Gospodarek, Eugenia
The mortality for dogs with severe immune-mediated hemolytic anemia (IMHA) is unacceptably high, and better immunosuppressive regimens are needed to increase survival. Understanding the basic immunology of the disease and the mechanisms of action of the available immunosuppressive therapies will help clinicians choose an appropriate immunosuppressive protocol. Prospective, randomized clinical studies must be conducted to evaluate the efficacy and safety of different combined immunosuppressive modalities to treat canine IMHA and improve patients' outcomes. PMID:19241356
Summary: We report the reversible MR findings in a 7-year-old girl with hemolytic uremic syndrome and mild encephalopathy. The splenium of the corpus callosum showed isointense to low signal intensity on T1-weighted images and high signal intensity on T2-weighted images, representing local edema. These findings returned to near normal on MR images obtained 1 week later. The patient recovered without
Hiroshi Ogura; Makoto Takaoka; Masashi Kishi; Masahide Kimoto; Takeshi Shimazu; Toshiharu Yoshioka; Hisashi Sugimoto
SummaryAutoimmune hemolytic anemias (AIHAs) may occur when specific autoantibodies react with red blood cell (RBC) antigens. Decompensated hemolysis and detectable autoantibodies against RBCs are classical findings. The autoantibodies preferentially react at 37 °C (warm autoantibodies). The majority of these autoantibodies are of the IgG class; IgM and IgA warm autoantibodies are less common. Roughly 50% of the patients have an
A. Salama; N. Ahrens; H. Kiesewetter
Atypical hemolytic uremic syndrome (HUS) frequently leads to end-stage renal failure and can relapse after transplantation. A 12-year-old girl presenting with familial atypical HUS with a factor H mutation was successfully transplanted 6 years after a first transplant that had failed because of immediate recurrent HUS. Prophylactic plasma exchange before and after transplantation was used. Two months after transplantation, concomitant with
Karolien H. Olie; Sandrine Florquin; Jaap W. Groothoff; René Verlaak; Lisa Strain; Timothy H. J. Goodship; Jan J. Weening; Jean-Claude Davin
Autosomal dominant hemolytic uremic syndrome (ADHUS) is a rare disorder with a poor prognosis that was considered to present\\u000a mainly in adults. Recurrent episodes of ADHUS were also thought to be uncommon. However, increasing reports suggest that children\\u000a are often affected, that recurrent episodes may occur pre-transplantation, and that post-transplant recurrences occur in about\\u000a 50% of cases. We describe the
B. S. Kaplan; M. B. Leonard
Three patients with hemolytic uremic syndrome (HUS) developed peripheral gangrene. Bilateral carotid artery thromboses occurred\\u000a in one of these patients after recovery from HUS. One patient had a long history of juvenile rheumatoid arthritis. In the\\u000a second patient, a flu-like illness preceded the onset of HUS. The third was one of two sisters, with the HUS appearing more\\u000a than 1
Bernard S. Kaplan; Clotilde D. Garcia; Russell W. Chesney; William E. Segar; Katia Giugno; Roberto Chem
BACKGROUND: Transfusion-related acute lung injury (TRALI) is the leading cause of transfusion-related mortality. The majority of the literature involves adult patients. The main objective of this study was to characterize the demographic features, clinical presentation, patient outcomes, and antibody profiles of TRALI patients reported to the Canadian Blood Service (CBS) and to assess similarities and differences between adult and pediatric TRALI cases. STUDY DESIGN AND METHODS: A retrospective review of cases of TRALI submitted to the CBS from 2001 to 2011 was performed. Information collected included recipient demographics, event details, blood component transfused, morbidity and mortality data, and donor antibody results. RESULTS: A total of 284 cases of definite, possible, or probable TRALI were reported. Six percent (n?=?17) occurred in children. There were no significant differences between pediatric or adult patients with TRALI. Most of the children who presented with TRALI were either teenagers or less than 1?year of age. The incident rate of reported TRALI cases in Canada per 100,000 red blood cell transfusions was estimated at 5.58 for children and 3.75 for adults. CONCLUSIONS: This study is the largest case series of reported TRALI cases in children. Crude modeling suggests that the incidence of TRALI in children is similar to that of adults. Although the numbers are small, there do not appear to be differences in presentation or outcome between adults and children with TRALI. TRALI is associated with significant morbidity and mortality and pediatricians need to consider this diagnosis in children who experience respiratory distress after transfusions. PMID:23763359
Lieberman, Lani; Petraszko, Tanya; Yi, Qi-Long; Hannach, Barbara; Skeate, Robert
Introduction Prediction of massive transfusion (MT) among trauma patients is difficult in the early phase of trauma management. Whole-blood thromboelastometry (ROTEM®) tests provide immediate information about the coagulation status of acute bleeding trauma patients. We investigated their value for early prediction of MT. Methods This retrospective study included patients admitted to the AUVA Trauma Centre, Salzburg, Austria, with an injury severity score ?16, from whom blood samples were taken immediately upon admission to the emergency room (ER). ROTEM® analyses (extrinsically-activated test with tissue factor (EXTEM), intrinsically-activated test using ellagic acid (INTEM) and fibrin-based extrinsically activated test with tissue factor and the platelet inhibitor cytochalasin D (FIBTEM) tests) were performed. We divided patients into two groups: massive transfusion (MT, those who received ?10 units red blood cell concentrate within 24 hours of admission) and non-MT (those who received 0 to 9 units). Results Of 323 patients included in this study (78.9% male; median age 44 years), 78 were included in the MT group and 245 in the non-MT group. The median injury severity score upon admission to the ER was significantly higher in the MT group than in the non-MT group (42 vs 27, P < 0.0001). EXTEM and INTEM clotting time and clot formation time were significantly prolonged and maximum clot firmness (MCF) was significantly lower in the MT group versus the non-MT group (P < 0.0001 for all comparisons). Of patients admitted with FIBTEM MCF 0 to 3 mm, 85% received MT. The best predictive values for MT were provided by hemoglobin and Quick value (area under receiver operating curve: 0.87 for both parameters). Similarly high predictive values were observed for FIBTEM MCF (0.84) and FIBTEM A10 (clot amplitude at 10 minutes; 0.83). Conclusions FIBTEM A10 and FIBTEM MCF provided similar predictive values for massive transfusion in trauma patients to the most predictive laboratory parameters. Prospective studies are needed to confirm these findings.
Strains of Helicobacter pylori, the bacterium associated with gastritis, peptic ulcer disease, and gastric cancer in humans, express different degrees of hemolysis on agar containing erythrocytes (RBC). Here we report the isolation and characterization of six recombinant clones from a genomic library of H. pylori ATCC 49503 that confer on Escherichia coli the ability to lyse sheep RBC. DNA hybridizations indicated no sequence homology among these hemolytic clones. Hybridization mapping of them to an ordered H. pylori cosmid library identified their separate chromosomal locations. One clone hybridized to two regions separated by approximately 200 kb. The specificities of the hemolytic activities of these clones were tested with RBC from humans, monkeys, cattle, horses, guinea pigs, rabbits, and chickens as well as with RBC from sheep. One clone conferred the ability to lyse RBC from five species, a second clone allowed the lysis of RBC from four of these species, three other clones allowed the lysis of RBC from three of these species, and the sixth clone allowed the lysis of RBC from just two species. We propose that some or all of the genes that confer these various hemolytic activities contribute to pathogen-host tissue interactions and that the different specificities seen here are important for H. pylori infections of humans of different genotypes or disease states.
Drazek, E S; Dubois, A; Holmes, R K; Kersulyte, D; Akopyants, N S; Berg, D E; Warren, R L
Hemolytic uremic syndrome (HUS) is a heterogeneous group of hemolytic disorders. Different terminologies have been described in HUS, which are as follows: (1) D+ HUS: Presentation with a preceding diarrhea; (2) typical HUS: D+ HUS with a single and self-limited episode; (3) atypical HUS (aHUS): Indicated those with complement dysregulation; (4) recurrent HUS: Recurrent episodes of thrombocytopenia and/or microangiopathic hemolytic anemia (MAHA) after improvement of hematologic abnormalities; and (5) familial HUS: Necessary to distinct synchronous outbreaks of D+ HUS in family members and asynchronous disease with an inherited risk factor. aHUS is one of the potential causes of end-stage renal disease (ESRD) in children. It has a high recurrence after renal transplantation in some genetic forms. Therefore, recognition of the responsible mechanism and proper prophylactic treatment are recommended to prevent or delay the occurrence of ESRD and prolong the length of survival of the transplanted kidney. A computerized search of MEDLINE and other databases was carried out to find the latest results in pathogenesis, treatment, and prevention of aHUS.
Nickavar, Azar; Sotoudeh, Kambiz
Hemolytic uremic syndrome (HUS) is a heterogeneous group of hemolytic disorders. Different terminologies have been described in HUS, which are as follows: (1) D+ HUS: Presentation with a preceding diarrhea; (2) typical HUS: D+ HUS with a single and self-limited episode; (3) atypical HUS (aHUS): Indicated those with complement dysregulation; (4) recurrent HUS: Recurrent episodes of thrombocytopenia and/or microangiopathic hemolytic anemia (MAHA) after improvement of hematologic abnormalities; and (5) familial HUS: Necessary to distinct synchronous outbreaks of D+ HUS in family members and asynchronous disease with an inherited risk factor. aHUS is one of the potential causes of end-stage renal disease (ESRD) in children. It has a high recurrence after renal transplantation in some genetic forms. Therefore, recognition of the responsible mechanism and proper prophylactic treatment are recommended to prevent or delay the occurrence of ESRD and prolong the length of survival of the transplanted kidney. A computerized search of MEDLINE and other databases was carried out to find the latest results in pathogenesis, treatment, and prevention of aHUS. PMID:23412906
Nickavar, Azar; Sotoudeh, Kambiz
Serratia marcescens is a common contaminant of contact lens cases and lenses. Hemolytic factors of S. marcescens contribute to the virulence of this opportunistic bacterial pathogen. We took advantage of an observed hyper-hemolytic phenotype of crp mutants to investigate mechanisms of hemolysis. A genetic screen revealed that swrW is necessary for the hyper-hemolysis phenotype of crp mutants. The swrW gene is required for biosynthesis of the biosurfactant serratamolide, previously shown to be a broad-spectrum antibiotic and to contribute to swarming motility. Multicopy expression of swrW or mutation of the hexS transcription factor gene, a known inhibitor of swrW expression, led to an increase in hemolysis. Surfactant zones and expression from an swrW-transcriptional reporter were elevated in a crp mutant compared to the wild type. Purified serratamolide was hemolytic to sheep and murine red blood cells and cytotoxic to human airway and corneal limbal epithelial cells in vitro. The swrW gene was found in the majority of contact lens isolates tested. Genetic and biochemical analysis implicate the biosurfactant serratamolide as a hemolysin. This novel hemolysin may contribute to irritation and infections associated with contact lens use.
Shanks, Robert M. Q.; Stella, Nicholas A.; Lahr, Roni M.; Wang, Shaoru; Veverka, Tara I.; Kowalski, Regis P.; Liu, Xinyu
A simple microassay and computer program are described for determining the erythrocyte hemolytic potency of drugs in vitro. This microassay is sensitive for both micro as well as macro ranges of hemoglobin concentration. An ELISA reader has been adapted to read erythrocyte lysis (hemolysis), which reduces the number and culture of replicates. A computer program was developed that calculates parameters such as C50 (concentration of drug causing 50% hemolysis), C100 (concentration of drug causing 100% hemolysis) and beta (slope of the curve) and graphically expresses the hemolytic patterns of various drugs simultaneously. The program can obtain optical densities directly from a 96-well plate ELISA reader by interfacing the microplate reader to the computer or by using a keyboard. This method is useful for screening a large number of hemolytic drugs and requires lower amounts of test compounds. It may also be applicable to quantitative functional assays, such as complement-mediated hemolysis and enumeration of antibody-secreting cells. The program can be obtained from the authors on request. PMID:7873185
Raghava, G P; Goel, A; Singh, A M; Varshney, G C
Background Cardiopulmonary adverse events after transfusion include acute lung injury (TRALI) and circulatory overload (TACO), which are potentially lethal and incompletely understood. Study Design and Methods To determine whether the incidence of TRALI and TACO was affected by leukoreduction we conducted a retrospective, before and after study of acute transfusion reactions for the seven years prior to and after introduction of universal leukoreduction in 2000, involving 778,559 blood components. Results Substantial decreases occurred in the rates of TRALI (?83%; from 2.8 cases per 100,000 components pre- to 0.48 post-universal leukoreduction; p=0.01), TACO (?49%; 7.4 to 3.8 cases per 100,000; p=0.03) and febrile reactions (?35%; 11.4 to 7.4 cases per 10,000; p<0.0001). The incidence of allergic reactions remained unchanged (7.0 per 100,000 pre- and post-universal leukoreduction). These outcomes were primarily attributable to decreased TRALI/TACO associated with RBC and platelet transfusions (?64%) with notably smaller decreases associated with FFP or cryoprecipitate transfusions (?29%). The incidence of TRALI/TACO after 28,120 washed red cell and 69,325 platelet transfusions was zero. Conclusion These data suggest novel hypotheses for further testing in animal models, in prospective clinical trials, and via the new US Hemovigilance System : (1) Is TACO or TRALI mitigated by leukoreduction? (2) Is the mechanism of TACO more complex than excessive blood volume? (3) Does washing mitigate TRALI and TACO due to platelet and RBC transfusions?
Blumberg, Neil; Heal, Joanna M.; Gettings, Kelly; Phipps, Richard P.; Masel, Debra; Refaai, Majed; Kirkley, Scott; Fialkow, L. Benjamin
Background.?Exchange transfusion (ET) has biologic plausibility as an adjunct to antimalarial drugs in treating severe malaria and has been used for decades despite limited evidence of its efficacy in improving survival. We examined the efficacy of ET as an adjunct treatment for severe malaria using US surveillance data and reviewed the literature to update recommendations. Methods.?Patients with severe malaria reported to the US national malaria surveillance system during 1985-2010 were matched, and survival outcomes were compared between patients receiving and not receiving ET. The literature review used search terms "severe malaria" and "exchange transfusion." Case reports and series, observational and case-control studies, and meta-analysis were included. Results.?One hundred one patients receiving ET were matched to 314 patients not receiving ET. There was no statistically significant association between ET and survival outcome (odds ratio, 0.84; 95% confidence interval, .44-1.60). We found 87 articles, mostly case reports or series, showing successful use of ET, likely reporting bias. There were 12 comparative studies, most of which were retrospective cohort studies, underpowered with no significant differences in survival. A previously published meta-analysis of 8 comparative studies found no significant survival differences. Adverse events were rarely reported but included acute respiratory distress syndrome, ventricular fibrillation, and hypotension. Conclusions.?Despite rapid parasite clearance times resulting from ET, there is no evidence for efficacy of ET as adjunctive therapy in severe malaria. Adjunct ET cannot be recommended. When rapidly acting antimalarials, specifically artemisinins, become more widely available, the biologic plausibility argument for ET will become less relevant. PMID:23800940
Tan, Kathrine R; Wiegand, Ryan E; Arguin, Paul M
Currently transfused cellular components of blood are not available in a sterile form and carry a small risk of transmitting viral and parasite diseases. Using phthalocyanines and red light, lipid enveloped viruses, e.g., HIV-1, can be inactivated in red blood cell concentrates (RBCC). Under conditions leading to virus sterilization the blood borne parasites Trypanosoma cruzi (Chagas disease) and Plasmodium falciparum (malaria) could be eliminated to undetectable levels (> 4 log10 kill). RBC damage during treatment could be avoided by increasing the light fluence rate to 80 mW/cm2, and by including the free radical scavenger glutathione and the vitamin E derivative Trolox during light exposure. Similar sterilization of platelet concentrates was achieved with the psoralen derivative AMT and UVA light. Platelet damage due to PUVA treatment was avoided by including the plant flavonoid rutin during irradiation. It is concluded that elimination of the risk of transmitting pathogens during blood transfusion is feasible with photochemical treatments.
Ben-Hur, Ehud; Margolis-Nunno, H.; Gottlieb, P.; Lustigman, S.; Horowitz, B.
Using a recently developed technique to extract jellyfish venom from nematocysts, the present study investigated the hemolytic activity of Cyanea nozakii Kishinouye nematocyst venom on chicken erythrocytes. Venom extract caused a significant concentration-dependent hemolytic effect. The extract could retain its activity at ?80°C but was unstable when kept at 4°C and ?20°C for 2 days. The hemolytic activity was inhibited
Jinhua Feng; Huahua Yu; Ronge Xing; Song Liu; Lin Wang; Shengbao Cai; Pengcheng Li
In this study, hemolytic activity of venom from the jellyfish Rhopilema esculentum Kishinouye and some factors affecting it were assayed. The HU50 of R. esculentum full venom (RFV) against chicken erythrocytes was 3.40?g\\/ml and a Hill coefficient value was 1.73 suggesting at least two molecules participated in hemolytic activity. The hemolytic activity of RFV was affected by some chemical and
Huahua Yu; Cuiping Li; Ronggui Li; Ronge Xing; Song Liu; Pengcheng Li
This editorial discusses the situation of administering blood to patients prior to radiotherapy in an attempt to increase tissue/tumor oxygen tension. The author believes that since the rate at which tumor cells consume oxygen is highly variable, the aim of achieving high cellular oxygen tension may be met better by maintaining a high blood perfusion rate. Blood volume can be maintained without relying on transfusion, and safer alternatives are available.
Hunt, T.K. (Univ. of California, San Francisco (USA))
Aims: To study the prevalence and severity of liver diseases of transfusion dependent thalassaemia major patients, and correlate the histological and biochemical changes of iron overload in liver with the peripheral blood markers.Method: Liver biopsy was performed to assess the histological changes and liver iron content (LIC).Results: One hundred patients were evaluated (median age 11.7 years, range 1.5–27). A total
C K Li; K W Chik; C W K Lam; K F To; S C H Yu; V Lee; M M K Shing; A Y K Cheung; P M P Yuen
Objective: To determine the incidence rate and the indications of red blood cell (RBC) transfusion in a pediatric intensive care unit (PICU).Patients and methods: A prospective observational cohort study of 303 consecutive patients was carried out in a multidisciplinary PICU in a tertiary care university hospital. All the patients were monitored daily over a three-month period. No interventions were done.Primary
F Gauvin; M Chaïbou; S Leteurtre; B Toledano; H Hume; F Proulx; P. C Hébert; A Martinot; F Leclerc; J Lacroix
In this study significantly prolonged canine renal allograft survival has been demonstrated after transfusion of 100 ml of third-party whole blood given peroperatively. Peroperative transfusions of third-party leukocyte-free blood or pure lymphocyte cell suspensions did not influence graft survival. Futhermore, no improvement in graft survival has been found after a peroperative transfuson of irradiated whole blood (2500 rad). These data suggest that delayed graft rejection after blood transfusions can only be expected after the administration of whole blood. The role of competent lymphocytes in whole blood is questionable, since a transfusion of irradiated whole blood in combination with nonirradiated lymphocytes did not lead to prolonged graft survival. Immunosuppression of the recipient directly after transfusion seems to be essential to induce the beneficial effect of blood transfusions. This has been demonstrated for a transfusion of whole blood 14 days before transplantation. A single transfusion of 100 ml of whole blood 14 days before transplantation could effectively prolong graft survival if immunosuppression with azathioprine and prednisone was started on the day of transfusion. No improvement in graft survival has been found with such a transfusion if preoperative immunosuppression has been omitted.
Van Der Linden, C.J.; Buurman, W.A.; Vegt, P.A.; Greep, J.M.; Jeekel, J.
A brief delay in clamping the umbilical cord results in a placental transfusion that supplies the infant with a major source of iron during the first few months of life. Cord circulation continues for several minutes after birth and placental transfusion results in approximately 30% more blood volume. Gravity influences the amount of placental transfusion that an infant receives. Placing the infant skin-to-skin requires a longer delay of cord clamping (DCC) than current recommendations. Uterotonics are not contraindicated with DCC. Cord milking is a safe alternative to DCC when one must cut the cord prematurely. Recent randomized controlled trials demonstrate benefits for term and preterm infants from DCC. The belief that DCC causes hyperbilirubinemia or symptomatic polycythemia is unsupported by the available research. Delay of cord clamping substantively increases iron stores in early infancy. Inadequate iron stores in infancy may have an irreversible impact on the developing brain despite oral iron supplementation. Iron deficiency in infancy can lead to neurologic issues in older children including poor school performance, decreased cognitive abilities, and behavioral problems. The management of the umbilical cord in complex situations is inconsistent between birth settings. A change in practice requires collaboration between all types of providers who attend births. PMID:22843002
Mercer, Judith S; Erickson-Owens, Debra A
Paul Holland began his career in transfusion medicine in 1963 as an assistant to Dr. Paul Schmidt in the Blood Bank at the National Institutes of Health (NIH). He served at the NIH for 20 years and retired in 1983 with the rank of Captain in the Public Health Service. He subsequently became the Medical Director/CEO of the Sacramento Medical Foundation Blood Center, now Blood Source, a position he held for the next 21 years. Paul Holland has authored/co-authored 265 articles, chapters and monographs, mostly concerning issues relating to either viral hepatitis or HIV. In addition to his research career, Paul was a very active educator, having contributed importantly to the development of many current thought leaders in transfusion medicine. His distinguished career also included important administrative roles in national and international organizations relevant to transfusion medicine. He also was the recipient of many honors and awards which has won him wide-spread renown and the respect of his many colleagues. PMID:23831199
McCarthy, Leo J
Blood transfusion safety has had a chequered history, and there are current and future challenges. Internationally, there is no clear consensus for many aspects of the provision of safe blood, although pan-national legislation does provide a baseline framework in the European Union. Costs are rising, and new safety measures can appear expensive, especially when tested against some other medical interventions, such as cancer treatment and vaccination programmes. In this article, it is proposed that a comprehensive approach is taken to the issue of blood transfusion safety that considers all aspects of the process rather than considering only new measures. The need for an agreed level of safety for specified and unknown risks is also suggested. The importance of providing care and support for those inadvertently injured as a result of transfusion problems is also made. Given that the current blood safety decision process often uses a utilitarian principle for decision making--through the calculation of Quality Adjusted Life Years--an alternative philosophy is proposed. A social contract for blood safety, based on the principles of 'justice as fairness' developed by John Rawls, is recommended as a means of providing an agreed level of safety, containing costs and providing support for any adverse outcomes. PMID:23171300
Franklin, I M
We have developed a miniaturized semiclosed cardiopulmonary bypass (CPB) circuit incorporating a centrifugal blood pump (TinyPump) with a volume of 5 ml. The current study was undertaken to evaluate the hemolytic performance of the TinyPump in comparison with the BioPump and to investigate the impact of different CPB circuit volumes on hemodilution, coagulation, and the inflammatory response. Twelve 1-week-old piglets (3.4 +/- 0.2 kg) were used. The circuit comprised a centrifugal pump, a membrane oxygenator, and a cardiotomy reservoir. Cardiopulmonary bypass was conducted with mild hypothermia at 150 ml/kg/min for 3 hours. Transfusion was not performed. Priming volume was 68 ml for the circuit with the TinyPump and 111 ml for the circuit with the BioPump. Although the TinyPump required higher speed, plasma free hemoglobin levels after CPB were not different between the groups. After CPB, the TinyPump group had a significantly higher hematocrit (27% +/- 3% vs. 23% +/- 3%) and lower platelet reduction rate, lower thrombin-antithrombin complex levels, and lower interleukin-6 levels. Better lung compliance with less water content was observed in the TinyPump group. The TinyPump maintained CPB with acceptable hemolysis and lower inflammatory responses. This miniaturized CPB circuit may make transfusion-free open heart surgery feasible in neonates and would help to prevent postoperative organ dysfunction. PMID:18043145
Ugaki, Shinya; Ishino, Kozo; Osaki, Satoru; Kotani, Yasuhiro; Honjo, Osami; Hoshi, Hideo; Yokoyama, Naoyuki; Ohuchi, Katsuhiro; Takatani, Setsuo; Sano, Shunji
Alloantibodies against HLA antigens can be reduced by applying leukodepletion to transfusions. Because the importance of immunological and nonimmunological causes of poor platelet transfusion results using leukodepleted transfusions is not clear, we conducted a prospective study in an unselected patient population receiving leukodepleted transfusions. In 97 patients with hematological malignancies, 181 random leukodepleted platelet transfusions were studied for immunological causes
M.-D. Levin; J. C. de Veld; B. van der Holt; M. B. van't Veer
\\u000a In this chapter, some of the special problems of red blood cell (RBC) transfusion in immunocompromised patients are discussed.\\u000a The transfusion therapy of some of these immunocompromised patients is dealt with specifically in later chapters (see Chapters 7, 8, and 12 on solid-organ transplant recipients, progenitor-cell transplant recipients, and chronically transfused\\u000a patients). Immunocompromised patients are obviously at risk for all
Christopher P. Stowell
Hemolytic uremic syndrome (HUS) is characterized by acute renal failure in children and is typically complicated with thrombocytopenia and hemolytic anemia. Although mouse models of HUS have been evaluated using Shiga toxin (STx) combined with or without lipopolysaccharide (LPS), no HUS model has been tested using purified outer membrane vesicles (OMVs) from STx-producing Escherichia coli (STEC) O157:H7. Accordingly, we investigated whether OMVs of STEC O157:H7 conveying STx2 and LPS can cause HUS-like symptoms in mice inoculated intraperitoneally. Three types of OMVs differing in LPS acylation status and STx2 amount were used to compare their ability to induce HUS-like symptoms. Native OMVs (nOMV) with fully hexa-acylated LPS caused HUS-like symptoms at 72-96 h after dually divided injections of 1 ?g nOMV per animal. However, elevated doses of modified OMVs (mOMV) carrying mainly penta-acylated LPS were required to induce similar HUS signs. Moreover, mitomycin-C-induced OMVs (mcOMV) that were enriched with STx2 induced HUS-like symptoms similar to those of nOMV at the same dose. These results suggest that the OMVs of STEC O157:H7 potentiated with STx2 and fully hexa-acylated LPS can be utilized for inducing HUS-like symptoms in mice and could be the causative material involved in the development of HUS. PMID:22029600
Kim, Sang-Hyun; Lee, Yong-Hoon; Lee, Sang-Ho; Lee, Sang-Rae; Huh, Jae-Won; Kim, Sun-Uk; Chang, Kyu-Tae
Transfusion of whole blood and some blood components may result in serious or fatal complications, among which hepatitis is most frequent (20,000 to 30,000 cases and 3,000 deaths a year). Although hepatitis B virus (HB Ag) sometimes is implicated in posttransfusion hepatitis, non-A non-B. virus(es) (hepatitis “C” virus) probably accounts for most posttransfusion hepatitis. Half of all blood transfusions may be unnecessary. Responsible transfusion practice requires use of appropriate blood components for which there is adequate justification. Transfusion of red blood cells should be given as packed cells in most instances and whole blood should seldom be used.
Johnston, D. Gordon
The production of chondroitin sulfatase, hyaluronidase, deoxyribonuclease, gelatinase, phosphatase, lecithinase, and hemolysins was examined in 95 strains of Propionibacterium acnes and four related species of anaerobic, respectively, microaerophilic coryneform bacteria (P. avidum, P. lymphophilum, P. granulosum, and Corynebacterium minutissimum). All enzymes could be demonstrated in at least one representative of the species tested. Those Propionibacterium species most frequently found in acne vulgaris lesions, i.e., P. acnes and P. granulosum, proved to be the most active organisms concerning the production of the enzymes tested. P. avidum, on the other hand, showed the highest rate of hemolytic activity.
This article analyzes the historic evolution of the Starr-Edwards prosthesis manufacture and its association to hemolysis. It describes also the information related to bioprosthesis and hemolysis. The mechanisms involved in mechanical hemolysis are discussed (turbulent flux, red cells trapping, construction material and autoimmunity). Reviews the pathophysiology and criteria for clinical and laboratory diagnosis of hemolysis. We describe the value of the quantitation of unconjugated bilirubin, free plasmatic hemoglobin, DHL and it's DHL1 iso enzyme, methemalbumin and urinary hemosiderin for the specific diagnosis of this entity. Finally we comment on the utility of bed rest, cellular maturity inductors, propranolol and sulfinpyrazone therapy for the control of the hemolytic process. PMID:2198853
Cervantes Escárcega, J L; Izaguirre Avila, R
Autoimmune hemolytic anemia (AIHA) is characterized by the presence of autoantibodies, most frequently of the IgG isotype, directed against erythrocyte surface antigens. The direct antiglobulin test (DAT) is the critical laboratory test for the diagnosis of AIHA, but is negative in 3-11% of cases. In these cases of DAT negative AIHA, a wider spectrum of clinical data including more specialized testing for erythrocyte autoantibodies may be required. We describe the unique and challenging case of an infant with corticosteroid-responsive, DAT negative AIHA, in which specialized gel card testing identified an isolated IgA autoantibody on the erythrocyte surface. PMID:21370419
McGann, Patrick T; McDade, Jenny; Mortier, Nicole A; Combs, Martha R; Ware, Russell E
BACKGROUND The mechanisms that underlie allergic transfusion reactions (ATRs) are not well characterized, but likely involve recipient, donor, and product factors. To assess product factors associated with ATRs, we investigated candidate mediators in apheresis platelet products associated with ATRs and controls. STUDY DESIGN AND METHODS Using bead-based and standard ELISA immunoassays, we tested supernatants from 20 consecutive apheresis platelet transfusions associated with ATRs and 30 control products for concentrations of mediators in 3 categories: acute inflammatory mediators, direct agonists of basophils and mast cells, and growth/priming factors of basophils and mast cells. RESULTS Median concentrations of the direct allergic agonists C5a, brain derived neurotrophic factor (BDNF), and CCL5 (RANTES) were 16.6%, 41.8%, and 13.9% higher, respectively, in the supernatant of apheresis platelet products that were most strongly associated with ATRs (P < 0.05 for each mediator). Other direct agonists (MIP-1?, MCP-1, eotaxin-1, IL-8) were similar between groups. Concentrations of acute inflammatory mediators and basophil growth/priming factors were also similar between groups (P > 0.2 for all associations). CONCLUSION The allergic agonists C5a, BDNF, and CCL5 may be mediators of ATRs in apheresis platelet products. Acute inflammatory proteins and basophil/mast cell growth and priming factors do not appear to be associated with apheresis platelet products that cause ATRs.
Savage, William J.; Savage, Jessica H.; Tobian, Aaron A.R.; Thoburn, Chris; Hamilton, Robert G.; Schroeder, John T.; Ness, Paul M.
We describe 3 cases of acute graft-versus-host (GVH) disease in patients with acute myeloid leukaemia following transfusions taken from non-HLA-identical healthy donors. The leucocyte transfusions were given because of severe bone marrow aplasia and granulocytopenia following leukaemia induction treatment. The first patient had an acute GVH reaction with an erythrodermia-like skin reaction all over and associated with severe abdominal cramping, enlarged liver and pathological liver function tests. The second patient had a relatively mild skin reaction and enlarged liver. Both died of severe pulmonary infection. The third patient also had a mild skin reaction and enlarged liver. He died of pulmonary embolism. The diagnosis of GVH of the latter 2 cases was made on skin biopsy. The autopsy samples revealed in all cases a heavy lymphocytic infiltration of the kidneys and liver portal area. Until more precise guidelines can be established, irradiation of blood cell products given to patients with neutropenia due to leukaemia induction treatment should be considered. PMID:6580718
Nikoskelainen, J; Söderström, K O; Rajamäki, A; Meurman, L; Korvenranta, H; Kalliomäki, J L; Toivanen, A
Recent interest in the health consequences of ricin as a weapon of terrorism has led us to investigate the effects of ricin on cells in vitro and in mice. Our previous studies showed that depurination of the 28S rRNA by ricin results in the inhibition of translation and the coordinate activation of the stress-activated protein kinases JNK and p38 MAPK. In RAW 264.7 macrophages, ricin induced the activation of ERK, JNK, and p38 MAPK, the accumulation of mRNA encoding tumor necrosis factor (TNF)-?, interleukin (IL)-1, the transcription factors c-Fos, c-Jun, and EGR1, and the appearance of TNF-? protein in the culture medium. Using specific inhibitors of MAPKs, we demonstrated the nonredundant roles of the individual MAPKs in mediating proinflammatory gene activation in response to ricin. Similarly, the intravenous administration of ricin to mice led to the activation of ERK, JNK, and p38 MAPK in the kidneys, and increases in plasma-borne TNF-?, IL-1?, and IL-6. Ricin-injected mice developed the hallmarks of hemolytic uremic syndrome, including thrombotic microangiopathy, hemolytic anemia, thrombocytopenia, and acute renal failure. Microarray analyses demonstrated a massive proinflammatory transcriptional response in the kidneys, coincidental with the symptoms of hemolytic uremic syndrome. Therapeutic management of the inflammatory response may affect the outcome of intoxication by ricin.
Korcheva, Veselina; Wong, John; Corless, Christopher; Iordanov, Mihail; Magun, Bruce
Background Fresh frozen plasma (FFP) is an effective therapy to correct for a deficiency of multiple coagulation factors during bleeding. In past years, use of FFP has increased, in particular in patients on the Intensive Care Unit (ICU), and has expanded to include prophylactic use in patients with a coagulopathy prior to undergoing an invasive procedure. Retrospective studies suggest that prophylactic use of FFP does not prevent bleeding, but carries the risk of transfusion-related morbidity. However, up to 50% of FFP is administered to non-bleeding ICU patients. With the aim to investigate whether prophylactic FFP transfusions to critically ill patients can be safely omitted, a multi-center randomized clinical trial is conducted in ICU patients with a coagulopathy undergoing an invasive procedure. Methods A non-inferiority, prospective, multicenter randomized open-label, blinded end point evaluation (PROBE) trial. In the intervention group, a prophylactic transfusion of FFP prior to an invasive procedure is omitted compared to transfusion of a fixed dose of 12 ml/kg in the control group. Primary outcome measure is relevant bleeding. Secondary outcome measures are minor bleeding, correction of International Normalized Ratio, onset of acute lung injury, length of ventilation days and length of Intensive Care Unit stay. Discussion The Transfusion of Fresh Frozen Plasma in non-bleeding ICU patients (TOPIC) trial is the first multi-center randomized controlled trial powered to investigate whether it is safe to withhold FFP transfusion to coagulopathic critically ill patients undergoing an invasive procedure. Trial Registration Trial registration: Dutch Trial Register NTR2262 and ClinicalTrials.gov: NCT01143909
In the same way that cry genes, coding for larvicidal delta endotoxins, constitute a large and diverse gene family, the cyt genes for hemolytic toxins seem to compose another set of highly related genes in Bacillus thuringiensis. Although the occurrence of Cyt hemolytic factors in B. thuringiensis has been typically associated with mosquitocidal strains, we have recently shown that cyt
ALEJANDRA GUERCHICOFF; ARMELLE DELECLUSE; CLARA P. RUBINSTEIN
The definition of anemia is controversial and varies with the sex, age, and ethnicity of the patient. Anemia afflicts half of hospitalized patients and most elderly hospitalized patients. Acute anemia in the operating room or intensive care unit is associated with increased morbidity as well as other adverse outcomes, including death. The risks of anemia are compounded by the added risks associated with transfusion of red blood cells, the most common treatment for severe anemia. The causes of anemia in hospitalized patients include iron deficiency, suppression of erythropoietin and iron transport, trauma, phlebotomy, coagulopathies, adverse effects of and reactions to medications, and stress-induced gastrointestinal bleeding. The types and causes of anemia and the increased health care utilization and costs associated with anemia and undetected internal bleeding are described. The potential benefits and risks associated with transfusion of red blood cells also are explored. Last, the strategies and new tools to help prevent anemia, allow earlier detection of internal bleeding, and avoid unnecessary blood transfusions are discussed. PMID:24186829
McEvoy, Michael T; Shander, Aryeh
Analysis of recently transfused patients is usually postponed to avoid spurious results because of contamination with donor's cells. However, little is known about the extent of this influence in routine molecular diagnostic tests. To elucidate this question, we tested a mix of blood samples from 2 ?-1-antitrypsin-deficient patients diagnosed as Pi*Z homozygous with 1 normal donor at 1:1, 1:10, 1:20, and 1:30 proportions. Human identification panel and Pi*Z allele detection were used to establish the detection limit of a blood mixture. Mixtures of 1:1 and 1:10 were easily detected with both techniques, whereas for 1:30, it was necessary to change the equipment settings to identify the mixture. Moreover, the heterozygous pattern observed for the mixtures on Pi*Z genotyping was weaker at this level of mixture. We further evaluated the degree of mixture detectable in 20 transfused patients who received 1 blood unit (concentrate of irradiated or nonirradiated red blood cells) using the human identification panel. Two days after the transfusion, the presence of the donor's markers was not detected, suggesting that after this time point the levels of admixture are below 1:30. The methods applied in the present study showed adequate sensitivity to identify alleles of the so-called "smaller population" of cells up to 3%, approximately. The same result was obtained in a "diagnostic situation," in which the blood mixture was submitted to a PCR-RFLP protocol to detect a mutation. PMID:23628825
Mardini, Ana C; Mayer, Fabiana Q; Rodenbusch, Rodrigo; Matte, Ursula; Saraiva-Pereira, Maria L
Two novel ADAMTS13 gene mutations in thrombotic thrombocytopenic purpura\\/hemolytic-uremic syndrome (TTP\\/HUS).BackgroundThrombotic thrombocytopenic purpura (TTP) and hemolytic-uremic syndrome (HUS) are now considered to be variants of one single syndrome called thrombotic thrombocytopenic purpura\\/hemolytic-uremic syndrome (TTP\\/HUS). Key features are thrombocytopenia, hemolytic anemia, and subsequently impaired function of different organs, especially the kidneys and the central nervous system (CNS). One possible reason is
CHRISTOPH LICHT; LUDWIG STAPENHORST; THORSTEN SIMON; ULRICH BUDDE; REINHARD SCHNEPPENHEIM; BERND HOPPE
BACKGROUND The hemoglobin threshold at which postoperative red-cell transfusion is warranted is controversial. We conducted a randomized trial to determine whether a higher threshold for blood transfusion would improve recovery in patients who had undergone surgery for hip fracture. METHODS We enrolled 2016 patients who were 50 years of age or older, who had either a history of or risk factors for cardiovascular disease, and whose hemoglobin level was below 10 g per deciliter after hip-fracture surgery. We randomly assigned patients to a liberal transfusion strategy (a hemoglobin threshold of 10 g per deciliter) or a restrictive transfusion strategy (symptoms of anemia or at physician discretion for a hemoglobin level of <8 g per deciliter). The primary outcome was death or an inability to walk across a room without human assistance on 60-day follow-up. RESULTS A median of 2 units of red cells were transfused in the liberal-strategy group and none in the restrictive-strategy group. The rates of the primary outcome were 35.2% in the liberal-strategy group and 34.7% in the restrictive-strategy group (odds ratio in the liberal-strategy group, 1.01; 95% confidence interval [CI], 0.84 to 1.22), for an absolute risk difference of 0.5 percentage points (95% CI, ?3.7 to 4.7). The rates of in-hospital acute coronary syndrome or death were 4.3% and 5.2%, respectively (absolute risk difference, ?0.9%; 99% CI, ?3.3 to 1.6), and rates of death on 60-day follow-up were 7.6% and 6.6%, respectively (absolute risk difference, 1.0%; 99% CI, ?1.9 to 4.0). The rates of other complications were similar in the two groups. CONCLUSIONS A liberal transfusion strategy, as compared with a restrictive strategy, did not reduce rates of death or inability to walk independently on 60-day follow-up or reduce in-hospital morbidity in elderly patients at high cardiovascular risk. (Funded by the National Heart, Lung, and Blood Institute; FOCUS ClinicalTrials.gov number, NCT00071032.)
Carson, Jeffrey L.; Terrin, Michael L.; Noveck, Helaine; Sanders, David W.; Chaitman, Bernard R.; Rhoads, George G.; Nemo, George; Dragert, Karen; Beaupre, Lauren; Hildebrand, Kevin; Macaulay, William; Lewis, Courtland; Cook, Donald Richard; Dobbin, Gwendolyn; Zakriya, Khwaja J.; Apple, Fred S.; Horney, Rebecca A.; Magaziner, Jay
This project is aimed at developing a cost-effective working environment for the transfusion medicine specialists of American Red Cross (ARC). In this project we are developing a multimedia-based consultation environment that uses Internet and teleconferencing to increase the quality of services and to replace currently used 800 telephone lines. Through the use of Internet/LAN/ISDN the physicians can share information and references while they discuss patient cases. A multimedia interface allows the physician to access data from the office and from the house. This paper discusses the approach, current status of the project and future plans to extend the approach to other areas of medicine.
Maitan, Jacek; Haley, Rebecca
Fractures of the femoral neck are common, and their incidence seems likely to increase. A prospective study in 1991 of 80 patients with such fractures suggested that not all need to be cross-matched preoperatively, a finding supported by the existing literature. At the same time, a survey of transfusion protocols in hospitals throughout the country suggested that much blood was being wasted daily in unnecessary cross-matching. This survey was repeated in 1995, and little appears to have changed. The implications of this are discussed.
Twin-to-twin transfusion syndrome (TTTS) is a severe complication of monochorionic (MC) twin pregnancies, characterized by the development of unbalanced chronic blood transfer from one twin, defined as donor twin, to the other, defined as recipient, through placental anastomoses. If left untreated, TTTS is associated with very high perinatal mortality and morbidity rates, due to a combination of fetal and/or obstetric complications. The reported prevalence is 10-15% of all MC twins, or about 1 in 2000 pregnancies. This consensus document reviews available evidence and offers practical guidance to clinicians by providing recommendations on various aspects concerning diagnosis and management of TTTS. PMID:21142846
Baschat, Ahmet; Chmait, Ramen H; Deprest, Jan; Gratacós, Eduard; Hecher, Kurt; Kontopoulos, Efficia; Quintero, Ruben; Skupski, Daniel W; Valsky, Dan V; Ville, Yves
Introduction Retrospective studies have demonstrated a potential survival benefit from transfusion strategies using an early and more balanced ratio between fresh frozen plasma (FFP) concentration and packed red blood cell (pRBC) transfusions in patients with acute traumatic coagulopathy requiring massive transfusions. These results have mostly been derived from non-head-injured patients. The aim of the present study was to analyze whether a regime using a high FFP:pRBC transfusion ratio (FFP:pRBC ratio >1:2) would be associated with a similar survival benefit in severely injured patients with traumatic brain injury (TBI) (Abbreviated Injury Scale (AIS) score, head ?3) as demonstrated for patients without TBI requiring massive transfusion (?10 U of pRBCs). Methods A retrospective analysis of severely injured patients from the Trauma Registry of the Deutsche Gesellschaft für Unfallchirurgie (TR-DGU) was conducted. Inclusion criteria were primary admission, age ?16 years, severe injury (Injury Severity Score (ISS) ?16) and massive transfusion (?10 U of pRBCs) from emergency room to intensive care unit (ICU). Patients were subdivided into patients with TBI (AIS score, head ?3) and patients without TBI (AIS score, head <3), as well as according to the transfusion ratio they had received: high FFP:pRBC ratio (FFP:pRBC ratio >1:2) and low FFP:pRBC ratio (FFP:pRBC ratio ?1:2). In addition, morbidity and mortality between the two groups were compared. Results A total of 1,250 data sets of severely injured patients from the TR-DGU between 2002 and 2008 were analyzed. The mean patient age was 42 years, the majority of patients were male (72.3%), the mean ISS was 41.7 points (±15.4 SD) and the principal mechanism of injury was blunt force trauma (90%). Mortality was statistically lower in the high FFP:pRBC ratio groups versus the low FFP:pRBC ratio groups, regardless of the presence or absence of TBI and across all time points studied (P < 0.001). The frequency of sepsis and multiple organ failure did not differ among groups, except for sepsis in patients with TBI who received a high FFP:pRBC ratio transfusion. Other secondary end points such as ventilator-free days, length of stay in the ICU and overall in-hospital length of stay differed significantly between the two study groups, but not when only data for survivors were analyzed. Conclusions These results add more detailed knowledge to the concept of a high FFP:pRBC ratio during early aggressive resuscitation, including massive transfusion, to decrease mortality in severely injured patients both with and without accompanying TBI. Future research should be conducted with a larger number of patients to prove these results in a prospective study.
Susceptibility of 64 beta-hemolytic streptococcal strains isolated from the patients with sore throat was studied by the method of serial dilutions in fluid nutrient medium (Konikov broth). Heterogenecity with respect to the sensitivity was investigated in 34 strains among separate populations of the microbes (10 to 15 in every strain). The MIC of benzylpenicillin, oxacillin and erythromycin ranged within 0.007--0.24 U/ml, 0.02--0.36 gamma/ml and 0.005--0.1 gamma/ml respectively. The MIC of benzylpenicillin with respect to separate populations most sensitive to it was 0.007--0.015 U/ml, while that with respect to the lease sensitive populations ranged from 0.015 to 0.24 U/ml. The respective values for oxacillin were 0.02--0.12 and 0.18--0.36 gamma/ml and those for erythromycin were 0.005--0.025 and 0.05--0.1 gamma/ml. Therefore, the beta-hemolytic streptococci isolated from the patients with sore throat were characterized by a rather high sensitivity to the antibiotics which was important precondition for their efficiency in treatment of the patients with the above disease. PMID:354509
Liashenko, Iu I; Khodyrev, A P
Bacillus subtilis produces an anionic bacteriocin called subtilosin A that possesses antibacterial activity against certain gram-positive bacteria. In this study, we uncovered a hemolytic mutant of B. subtilis that produces an altered form of subtilosin A. The mutant bacteriocin, named subtilosin A1, has a replacement of threonine at position 6 with isoleucine. In addition to the hemolytic activity, subtilosin A1 was found to exhibit enhanced antimicrobial activity against specific bacterial strains. The B. subtilis albB mutant that does not produce a putative immunity peptide was more sensitive to both subtilosin A and subtilosin A1. A spontaneous suppressor mutation of albB that restored resistance to subtilosin A and subtilosin A1 was obtained. The sbr (subtilosin resistance) mutation conferring the resistance is not linked to the sboA-alb locus. The sbr mutation does not increase the resistance of B. subtilis to other cell envelope-targeted antimicrobial agents, indicating that the mutation specifically confers the resistance to subtilosins. The findings suggest possible bioengineering approaches for obtaining anionic bacteriocins with enhanced and/or altered bactericidal activity. Furthermore, future identification of the subtilosin-resistant mutation could provide insights into the mechanism of subtilosin A activity. PMID:19633086
Huang, Tai; Geng, Hao; Miyyapuram, Venugopal R; Sit, Clarissa S; Vederas, John C; Nakano, Michiko M
Bacillus subtilis produces an anionic bacteriocin called subtilosin A that possesses antibacterial activity against certain gram-positive bacteria. In this study, we uncovered a hemolytic mutant of B. subtilis that produces an altered form of subtilosin A. The mutant bacteriocin, named subtilosin A1, has a replacement of threonine at position 6 with isoleucine. In addition to the hemolytic activity, subtilosin A1 was found to exhibit enhanced antimicrobial activity against specific bacterial strains. The B. subtilis albB mutant that does not produce a putative immunity peptide was more sensitive to both subtilosin A and subtilosin A1. A spontaneous suppressor mutation of albB that restored resistance to subtilosin A and subtilosin A1 was obtained. The sbr (subtilosin resistance) mutation conferring the resistance is not linked to the sboA-alb locus. The sbr mutation does not increase the resistance of B. subtilis to other cell envelope-targeted antimicrobial agents, indicating that the mutation specifically confers the resistance to subtilosins. The findings suggest possible bioengineering approaches for obtaining anionic bacteriocins with enhanced and/or altered bactericidal activity. Furthermore, future identification of the subtilosin-resistant mutation could provide insights into the mechanism of subtilosin A activity.
Huang, Tai; Geng, Hao; Miyyapuram, Venugopal R.; Sit, Clarissa S.; Vederas, John C.; Nakano, Michiko M.
Atypical hemolytic uremic syndrome (aHUS) is a major thrombotic microangiopathy (TMA). A TMA is recognized by the laboratory signs of microangiopathic hemolysis, as indicated by schistocytes, elevated lactate dehydrogenase, low haptoglobin, and low hemoglobin, plus thrombocytopenia and accompanying signs and symptoms of organ system involvement. aHUS results from chronic, uncontrolled activity of the alternative complement pathway. In most patients, this defect is related to a genetic deficiency in one or more soluble and/or membrane-bound complement regulatory proteins. Complement factor H is most frequently implicated. Clinically, aHUS is often indistinguishable from the other TMAs: Shiga toxin–producing Escherichia coli (STEC) hemolytic uremic syndrome and thrombotic thrombocytopenic purpura (TTP). TTP and aHUS are associated with high morbidity and mortality. aHUS has a distinct pathology from TTP. In nearly all patients, aHUS can be distinguished from TTP on the basis of an ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) enzyme activity measurement. It is essential that aHUS and TTP be differentiated quickly, as they require markedly divergent treatments. The standard treatment for TTP is plasma exchange, a therapy that has no role for patients with a diagnosis of aHUS established by ADAMTS13 activity levels. PMID:23187605
Pathologic thrombosis is a major cause of mortality. Hemolytic-uremic syndrome (HUS) features episodes of small-vessel thrombosis resulting in microangiopathic hemolytic anemia, thrombocytopenia and renal failure. Atypical HUS (aHUS) can result from genetic or autoimmune factors that lead to pathologic complement cascade activation. Using exome sequencing, we identified recessive mutations in DGKE (encoding diacylglycerol kinase ?) that co-segregated with aHUS in nine unrelated kindreds, defining a distinctive Mendelian disease. Affected individuals present with aHUS before age 1 year, have persistent hypertension, hematuria and proteinuria (sometimes in the nephrotic range), and develop chronic kidney disease with age. DGKE is found in endothelium, platelets and podocytes. Arachidonic acid-containing diacylglycerols (DAG) activate protein kinase C (PKC), which promotes thrombosis, and DGKE normally inactivates DAG signaling. We infer that loss of DGKE function results in a prothrombotic state. These findings identify a new mechanism of pathologic thrombosis and kidney failure and have immediate implications for treating individuals with aHUS. PMID:23542698
Lemaire, Mathieu; Frémeaux-Bacchi, Véronique; Schaefer, Franz; Choi, Murim; Tang, Wai Ho; Le Quintrec, Moglie; Fakhouri, Fadi; Taque, Sophie; Nobili, François; Martinez, Frank; Ji, Weizhen; Overton, John D; Mane, Shrikant M; Nürnberg, Gudrun; Altmüller, Janine; Thiele, Holger; Morin, Denis; Deschenes, Georges; Baudouin, Véronique; Llanas, Brigitte; Collard, Laure; Majid, Mohammed A; Simkova, Eva; Nürnberg, Peter; Rioux-Leclerc, Nathalie; Moeckel, Gilbert W; Gubler, Marie Claire; Hwa, John; Loirat, Chantal; Lifton, Richard P
Autoimmune hemolytic anemia (AIHA) is an infrequent group of diseases defined by autoantibody mediated red blood cell destruction. Correct diagnosis and classification of this condition are essential to provide appropriate treatment. AIHA is divided into warm and cold types according to the characteristics of the autoantibody involved and by the presence of an underlying or associated disorder into primary and secondary AIHA. Due to its low frequency, treatment for AIHA is largely based on small prospective trials, case series, and empirical observations. This review describes in detail the different treatment approaches for autoimmune hemolytic anemia. Warm antibody type AIHA should be treated with steroids, to which most patients respond, although relapse can occur and maintenance doses are frequently required. Splenectomy is an effective second line treatment and can provide long-term remission without medication. Rituximab is a useful alternative for steroid refractory patients, those requiring high maintenance doses and unfavorable candidates for surgery. Promising therapeutic modifications with this monoclonal antibody are emerging including drug combinations, lower doses, and long-term use. Primary cold agglutinin disease has been recognized as having a lymphoproliferative monoclonal origin. It is unresponsive to both steroids and splenectomy. Rituximab is currently the best therapeutic alternative for this condition, and several treatment regimens are available with variable responses. PMID:23689532
Jaime-Pérez, José Carlos; Rodríguez-Martínez, Marisol; Gómez-de-León, Andrés; Tarín-Arzaga, Luz; Gómez-Almaguer, David
We report the inactivation of the third component of complement (C3) by hydroxylamine. C3 hemolytic and covalent binding activities decline with identical kinetics, demonstrating a direct correlation between the two activities. We conclude that covalent, surface-bound C3b is hemolytically active. The inactivation of C3 is first order with respect to hydroxylamine. We also studied C3 inactivation with [14C]methylamine. The inactivation corresponds quantitatively with the labeling of C3 in the C3d domain. The data obtained support the following hypothesis: there is an internal thioester within C3 which becomes highly reactive on activation to C3b, and C3b binds to receptive surfaces by transfer of the acyl function of the thioester to a hydroxyl group on the receptive surface. This proposed model for the reaction of C3 with receptive surfaces also applies to C4, which binds to membrane surfaces covalently and is able to be inactivated by hydroxylamine and methylamine. C5, on the other hand, is not inactivated by treatment with the amines. Images
Law, S K; Lichtenberg, N A; Levine, R P
In order to detect immunoglobulin (Ig)A and IgG antibodies to Escherichia coli-secreted protein B in sera of children infected with Shiga toxin-producing Escherichia coli, an enzyme-linked immunosorbent assay was developed. The assay was tested using acute sera from 40 children with diarrhea-associated hemolytic uremic syndrome compared with 238 sera obtained from pediatric controls. Two cut-off values were used for children
A.-C. Sjögren; J. B. Kaper; A. Caprioli; D. Karpman
Background. A retrospective study carried out on medical records of transfused patients in our hospital in 2002 revealed that manual identification procedures were insufficient to offer satisfactory traceability. The aim of this study was to assess adequacy of transfusion traceability and compliance with proper identification procedures after introducing an electronic identification system (EIS) for transfusion safety. Materials and methods. The chosen EIS (Gricode®) was set up. Traceability was calculated as the percentage of empty blood units used returned to the Transfusion Service, compared to the number of supplied units. Compliance in the Transfusion Service was calculated as the percentage of electronic controls from dispatch of blood components/transfusion request performed, compared to the total number of transfused units. Compliance in the ward was calculated as the percentage of electronic controls from sample collection/transfusion performed, compared to the total number of samples collected. Results. This retrospective study showed that only 48.0% of the medical records were free of inaccuracies. After the implementation of the EIS (2005–2008), traceability was always above 99%. Percentage of monthly compliance from 2006 to 2008 was always above 93%, showing a significant trend to increase (p<0.05). The mean compliance in this period was higher in the Transfusion Service (97.8±0.7 SD) than in the ward (94.9±2.4 SD; p<0.001). Compliance in the ward was lowest when the system was first implemented (87.9% in April 2006) after which it progressively increased. No errors in ABO transfusions were registered. Conclusion. After implementation of the EIS, traceability and compliance reached very high levels, linked to an improvement in transfusion safety.
Uriz, Maria Jose; Antelo, Maria Luisa; Zalba, Saioa; Ugalde, Nazaret; Pena, Esther; Corcoz, Andrea
Morphologic and biochemical changes occur during red cell storage prior to product expiry, and these changes may hinder erythrocyte viability and function following transfusion. Despite a relatively large body of literature detailing the metabolic and structural deterioration that occurs during red cell storage, evidence for a significant detrimental clinical effect related to the transfusion of older blood is relatively less
Marianne J Vandromme; Gerald McGwin Jr; Jordan A Weinberg
Objective: To see whether there was a link between blood transfusion and lipid peroxidation as measured by urinary malondialdehyde (MDA) concentration in preterm infants. Methods: Urine samples were collected before and after blood transfusions in preterm infants. Twenty blood transfusion episodes were studied in 12 infants (some infants were studied on more than one occasion). Twenty two infants who had not received a transfusion were used as controls. All infants were preterm and less than 1500 g birth weight. Urinary MDA was measured using a thiobarbituric acid assay and expressed as nmol/mg creatinine. Results: The median (interquartile range) urinary MDA concentration before transfusion was 9.1 (6.4–12.6) nmol/mg, and was not significantly different from that in the 22 non-transfused infants (11.3 (7.3–15.6) nmol/mg). There was a significant increase 24 hours after transfusion to 14.6 (7.3–23.7) nmol/mg, but it decreased to 10.1 (6.6–15.4) nmol/mg when measured a median (range) of 6 (3–9) days later. Conclusions: Blood transfusions were associated with evidence of increased lipid peroxidation. If lipid peroxidation contributes to the pathogenesis of retinopathy of prematurity and chronic lung disease, these results suggest an explanatory mechanism.
Wardle, S; Drury, J; Garr, R; Weindling, A
Massive haemorrhage in elective surgery can be either anticipated (e.g. organ transplantation) or unexpected. Management requires early recognition, securing haemostasis and maintenance of normovolaemia. Transfusion management involves the transfusion of packed red cells, platelet concentrates and plasma (fresh frozen plasma and cryoprecipitate). Blood product support should be based on clinical judgment and be guided by repeated laboratory tests of coagulation.
Wendy N Erber
Blood transfusion in a modern sense means the transfusion of red cells, when necessary supplemented by other components. The demand for plasma and plasma fractions and for platelets for therapeutic use has had an influence on the technique for preparing red cells. Automated devices have made it possible to perform collection as well as separation under more standardized conditions. Improved
Claes F. Högman
Blood transfusion services were poorly developed until the mid 1980s in most of sub-Saharan Africa, and were unable to provide adequate supplies of blood with acceptable safety. The pandemic of HIV was recognized seroepidemiologically from 1985 onwards. Blood transfusion was contributing from 10 to 15% to transmission in Africa. Groups at highest risk are children with malaria and anaemia, women
Alan F. Fleming
This paper examines the views of Jehovah's Witnesses in regards to their refusal of blood transfusions for themselves and their children. After setting out the legal framework society presently has in place for dealing with such refusals, the paper reviews the ethics literature that justifies the intervention by the State to force the transfusion of Jehovah's Witness children. It is
SummaryAutologous blood transfusion plays a significant part in hemotherapy. This relates not only to the legal stipulations as established in the Transfusion Act and handed down through court decisions but is also true under therapeutic aspects where many meaningful approaches towards autologous procedures are being made. Over and beyond that, autologous blood is a contribution towards self-sufficiency in blood and
F. von Auer
Objective: To evaluate the management of hydropic fetuses, due to rhesus isoimmunization, with fetal intrauterine intravascular transfusions. Material and Methods: This is a retrospective analysis of 18 rhesus-negative pregnant women presenting at our hospital with fetal hydrops during a 7-year period. All cases were managed with serial intrauterine intravascular transfusions with the goal of delivery by cesarean section beyond 33
Spiros Mesogitis; George Daskalakis; Athanasios Pilalis; Nikolaos Papantoniou; Aris Antsaklis
Objective To analyze the characteristics of patients who needed a blood transfusion due to epistaxis-caused anemia and to define potential risk factors. Design Retrospective cohort study. Setting A total cohort of 591 epistaxis patients, prospectively included between March 2007 and April 2008 at the ENT department of the University Hospital of Zurich, was evaluated concerning the need for blood transfusions. Methods The clinical charts and medical histories of these patients were evaluated. Main outcome measures Common parameters that increase the risk for severe anemia due to epistaxis. Results Twenty-two patients required blood transfusions due to their medical condition. 22.7% suffered from traumatic nosebleeds. Another 27.3% had a known medical condition with an increased bleeding tendency. These proportions were significantly higher than in the group of patients without need of blood transfusion. The odds ratio for receiving a blood transfusion was 14.0 in patients with hematologic disorders, 4.3 in traumatic epistaxis and 7.7 in posterior bleeders. The transfusion-dependent epistaxis patients suffered significantly more often from severe posterior nosebleeds with the need for a surgical therapeutic approach. Conclusions Patients with severe nosebleeds either from the posterior part of the nose or with known hematologic disorders or traumatic epistaxis should be closely monitored by blood parameter analyses to evaluate the indication for hemotransfusion. The acronym THREAT (Trauma, Hematologic disorder, and REAr origin of bleeding ? Transfusion) helps to remember and identify the factors associated with an increased risk of receiving blood transfusion.
Background: Several infectious diseases have been found to be associated with tattooing, including some transfusion transmitted diseases (TTDs). Information on tattooing has been included in screening interviews of prospective blood donors and may be a reason for deferral.Methods: Review of articles identified through MEDLINE (and other computerized data bases) using medical subject heading (MeSH) terms and textwords for “tattooing,” “transfusion,”
Sérgio de A. Nishioka; Theresa W. Gyorkos
Acute pharyngitis is a frequent pediatric consultation, being group A beta-hemolytic Streptococcus (GABHS) the main bacterial etiology. GABHS screening is controversial in children less than 5 years, because of the low frequency historically reported. Nevertheless, 24% prevalence was recently estimated in this group. The aim of this study was to estimate the GABHS frequency in symptomatic children less than 15 years, for which we performed a retrospective, cross sectional study, to evaluate its presence in the throat of 6691 patients, during 2010. The maximum frequency was observed in children from 5 to 11 years. For children aged 3 and 4, frequency was estimated at 36%, thus seeming to be mandatory to do the screening, as it is done in older children. In younger children, the epidemiology of each patient should be considered before asking for diagnostic tests. PMID:23224310
Tellechea, Ana L; Salvo, María G; Méndez, José H; Cavagnari, Brian M
Every year in France, approximately one hundred children, aged from 6 months to 3 years, develop hemolytic uremic syndrome (HUS) secondary to Shiga toxin-producing Escherichia coli (STEC) infection, mostly the O157:H7 serotype. Except during outbreaks, STEC-HUS is less frequent in adults. The main route of transmission is the consumption of undercooked ground beef or unpasteurized dairy products, or household contacts with STEC diarrhea. Mortality is 1 to 2%. Half of patients require dialysis at the acute phase, the majority will recover normal renal function but approximately 30% will suffer renal sequelae. The risk of sequelae is important if the duration of anuria has been more than 5 days. Treatment of STEC-HUS is mostly supportive. The efficacy of plasma exchanges is not demonstrated and that of eculizumab, a complement blocker, especially in case of central nervous system or cardiac involvement, uncertain. Prevention of STEC-HUS relies on simple methods, often unknown of parents of young children, with the French general population being scarcely informed. However, the surveillance of STEC-infections/HUS by the Institut de veille sanitaire and the Reference Center for E. coli infections allows the early detection of outbreaks and their source of contamination. This prevents the emergence of new cases by withdrawing the suspected food from the market and diffusing the information to the population (return of suspected food). PMID:23457821
HUS is characterized by hemolytic anemia, thrombocytopenia, and acute renal failure. While "typical" HUS is usually associated with Shiga toxin-producing Escherichia coli infections and recovers in the majority of cases, aHUS is caused by mutations of complement components or antibodies against CFH leading to uncontrolled activation of alternative complement pathway and often to ESRD. Recently, THBD gene mutations have been reported in aHUS. Theoretically, the risk of disease recurrence after renal transplantation should be low because THBD is primarily a membrane-bound protein expressed by endothelial cells; however, a small proportion of THBD is present as a soluble form in plasma. We report the case of a 19-yr-old man with aHUS secondary to a THBD mutation that relapsed twice after two renal transplantations performed 12 yr apart. Despite successful control of HUS with plasma exchange and eculizumab after the second transplantation, the graft was ultimately lost due to severe steroid-resistant cellular rejection. The present report suggests that THBD mutations may favor-relapse of aHUS after renal transplantation. PMID:24118826
Sinibaldi, Serena; Guzzo, Isabella; Piras, Rossella; Bresin, Elena; Emma, Francesco; Dello Strologo, Luca
From 1982 to 1990, 300 adults received liver transplants in Birmingham UK with a median intraoperative blood transfusion rate of 23.5 units for the first 50 patients falling to 8 units for the last 50. The major factors in the reduction of blood usage were the experience of the team, the use of venovenous bypass and the use of an argon beam coagulator. Univariate analysis of preoperative factors in an attempt to predict patients at risk of excessive intraoperative transfusion showed that levels of serum sodium, urea, creatinine, haemoglobin, patient weight and the presence of ascites were significantly related to the quantity of blood transfused, although stepwise discriminant analysis showed that only blood urea and platelet count had an independent association with transfusion. The final model was poorly predictive of intraoperative transfusion requirements. Technical factors rather than patient-related factors are more important in the control of intraoperative bleeding in newly established transplant programmes. PMID:8215151
Deakin, M; Gunson, B K; Dunn, J A; McMaster, P; Tisone, G; Warwick, J; Buckels, J A
The immunologic risk associated to erythrocyte transfusions is bound to the polymorphism of blood group systems and to the respect of blood transfusion regulations. The results of three studies are presented, which were carried out respectively by the French Society of Blood Transfusion, the National Institute of Blood Transfusion and the National Haemovigilance Network. Two hundred and twenty-seven cases of immunologic accidents are analysed using the Kaplan's interpretation model and the traditional method of process analysis. The results show three critical factors in the occurrence of this type of incident: the relevance of the clinical examinations prescribed, the way in which the biological results are taken into account, and the relationship/exchange of information between private and public hospitals, and blood transfusion centers. PMID:10730341
Rouger, P; Le Pennec, P Y; Noizat-Pirenne, F
Sickle cell intrahepatic cholestasis (SCIC) is a rare complication of sickle cell anemia, characterized by marked hyperbilirubinemia and acute hepatic failure with an often fatal course. However, the few reported adult cases that were treated with exchange transfusion had a favorable outcome. We herein describe a 48-year-old African-American man with hemoglobin S/B thalassemia and previously treated hepatitis C with compensated cirrhosis, who presented with a total bilirubin of 59.7 mg/dL and direct bilirubin of 43.6 mg/dL in the absence of choledocholithiasis. Despite an exchange transfusion and aggressive packed red blood cell transfusions, which successfully decreased the hemoglobin S levels to <15%, he perished from progressive hepatic and renal failure. Autopsy demonstrated extensive intrahepatocellular and intracanalicular cholestasis in a background of cirrhosis. Our case suggests that poor prognostic factors for adult SCIC patients treated with exchange transfusion may include older age and underlying hepatic disease. Images Figure 2
Costa, Daniel B.; Miksad, Rebecca A.; Buff, Michael S.; Wang, Yihong; Dezube, Bruce J.
Haemorrhage can be a lethal complication of severe acute pancreatitis. Management includes identification and control of the source of bleeding and supportive therapy such as blood transfusion. Individuals who refuse transfusion on the grounds of religious belief can provide a further major challenge. The management in these individuals can be focused from the outset with a strategy that aims to avert anaemia and transfusion. This article reports a case of severe acute pancreatitis in a woman of the Jehovah's Witness faith. The episode was complicated by infected pancreatic necrosis requiring surgical intervention. Careful strategic planning is critical to the management of severe acute pancreatitis in patients of the Jehovah's Witness faith. In this case, acute pancreatitis complicated by infected necrosis was successfully managed by the use of preoperative erythropoietin, venesection using paediatric blood vials, meticulous intraoperative attention to haemostasis and the use of adjunctive intraoperative techniques such as argon diathermy. PMID:16236094
Jamdar, S; Siriwardena, A K
In this study, hemolytic activity of venom from the jellyfish Rhopilema esculentum Kishinouye and some factors affecting it were assayed. The HU(50) of R. esculentum full venom (RFV) against chicken erythrocytes was 3.40 microg/ml and a Hill coefficient value was 1.73 suggesting at least two molecules participated in hemolytic activity. The hemolytic activity of RFV was affected by some chemical and physical factors such as divalent cations, EDTA, (NH(4))(2)SO(4), pH and temperature. In the presence of Mg(2+), Cu(2+), Zn(2+), Fe(2+), Ca(2+) (>or=2 mM), Mn(2+) ((>or=1 mM), EDTA ((>or=2 mM) and (NH(4))(2)SO(4), the hemolytic activity of RFV was reduced. RFV had strong hemolytic activity at the pH 6-10 and the hemolytic ratios were 0.95-1.19. Hemolytic activity was temperature-sensitive and when RFV was pre-incubated at temperatures over 40 degrees C, it was sharply reduced. PMID:17306433
Yu, Huahua; Li, Cuiping; Li, Ronggui; Xing, Ronge; Liu, Song; Li, Pengcheng
Summary Background and objectives Atypical hemolytic uremic syndrome is a disease associated with mutations in the genes encoding the complement regulators factors H and I. In addition, factor H autoantibodies have been reported in ?10% of patients with atypical hemolytic uremic syndrome. This study searched for the presence of factor I autoantibodies in atypical hemolytic uremic syndrome. Design, setting, participants, & measurements This study screened 175 atypical hemolytic uremic syndrome patients for factor I autoantibodies using ELISA with confirmatory Western blotting. Functional studies using purified immunoglobulin from one patient were subsequently undertaken. Results Factor I autoantibodies were detected in three patients. In one patient with a high titer of autoantibody, the titer was tracked over time and was found to have no association with disease activity. This study found evidence of an immune complex of antibody and factor I in this patient, but purified IgG, isolated from current serum samples, had only a minor effect on fluid phase and cell surface complement regulation. Genetic analysis of the three patients with factor I autoantibodies revealed that they had two copies of the genes encoding factor H–related proteins 1 and 3 and therefore, did not have a deletion commonly associated with factor H autoantibodies in atypical hemolytic uremic syndrome. Two patients, however, had functionally significant mutations in complement factor H. Conclusions These findings reinforce the concept of multiple concurrent risk factors being associated with atypical hemolytic uremic syndrome but question whether autoantibodies per se predispose to atypical hemolytic uremic syndrome.
Kavanagh, David; Pappworth, Isabel Y.; Anderson, Holly; Hayes, Christine M.; Moore, Iain; Hunze, Eva-Maria; Bennaceur, Karim; Roversi, Pietro; Lea, Susan; Strain, Lisa; Ward, Roy; Plant, Nick; Nailescu, Corina; Goodship, Timothy H. J.
We have studied the megakaryocytopoietic response in rats to acute thrombocytopenia induced by exchange transfusion of platelet-poor blood. Our analysis included serial determinations of peripheral blood counts, the size and number of megakaryocytes in sections of humeral marrow, the numbers of megakaryocytic (CFU-Meg) and granulocyte-macrophage (CFU-GM) colony-forming cells in marrow and spleen, and the proportion of CFU-Meg and CFU-GM in DNA synthesis. With exchange transfusion, the platelet count fell to 11% of the control value (101,000 +/- 49,000/mm3; mean +/- SD) and returned to normal by day 3; rebound thrombocytosis (peak 1,720,000 +/- 246,000/mm3) was observed on days 4 and 5. The average size of marrow megakaryocytes increased significantly on days 2 and 3 compared with normal (p less than 0.01), but the numbers of recognizable megakaryocytes did not change through day 5. The numbers of splenic CFU-Meg and CFU-GM increased significantly (p less than 0.05) on days 2-4 and on day 2, respectively, after the exchange; however, the numbers of marrow progenitors, which account for over 95% of total body progenitors, remained unchanged throughout the duration of the study. The proportion of CFU-Meg in DNA synthesis (mean +/- SD) increased from a baseline value of 17% +/- 4% to 33% +/- 11% (p less than 0.02) and 35% +/- 6% (p less than 0.001) on days 1 and 2, respectively, and returned to control values thereafter. There were no changes in the cell cycle activity of CFU-GM. Thus, acute selective thrombocytopenia induced by exchange transfusion causes an enlargement of marrow megakaryocytes and an increase in the fraction of CFU-Meg in cell cycle. These changes, occurring in the absence of immunologically mediated events, are the direct result of lowered platelet count. PMID:4054245
Kimura, H; Segal, G M; Lee, M Y; Adamson, J W
Group B ? -hemolytic Streptococcus (GBS) is a major pathogen affecting newborns. We have investigated the molecular mechanism underlying the respiratory distress induced in sheep after intravenous injection of a toxin produced by this organism. The pathophysiological response is characterized by pulmonary hypertension, followed by granulocytopenia and increased pulmonary vascular permeability to protein. 31P NMR studies of GBS toxin and model components before and after reductive alkaline hydrolysis demonstrated that phosphodiester residues are an integral part of the GBS toxin. Reductive alkaline treatment cleaves phosphate esters from secondary and primary alcohols and renders GBS toxin nontoxic in the sheep model and inactive as a mediator of elastase release in vitro from isolated human granulocytes. We propose that the interaction of cellular receptors with mannosyl phosphodiester groups plays an essential role in the pathophysiological response to GBS toxin.
Hellerqvist, Carl G.; Sundell, Hakan; Gettins, Peter
We report a rare case of autoimmune hemolytic anemia (AIHA) complicated by idiopathic interstitial pneumonia (IIP). A sixty-year-old man was diagnosed as having IIP in January 2009. In March, when he was hospitalized for the introduction of home oxygen therapy, severe anemia was detected. Based on the findings showing elevated levels of lactate dehydrogenase and indirect bilirubin, a decreased level of haptoglobin, positive Coombs test, and splenomegaly, a diagnosis of AIHA was made. Although anti-DNA antibody was found, diagnostic criteria for systemic lupus erythematosus and other collagen diseases were not fulfilled. Therefore, we concluded that AIHA coexisted with IIP. Treatment with prednisolone led to improvement of both AIHA and IIP. There has not been any exacerbation even after a gradual reduction of prednisolone to 7.5 mg/day. Coexistence of AIHA and IIP is rare, and accumulation of case reports is needed to gain a better understanding of this condition. PMID:21378476
Fukuda, Kuniyoshi; Yokoyama, Yasuhisa; Kamada, Yuhei; Taoka, Kenichi; Suzukawa, Kazumi; Chiba, Shigeru
Several case reports have described immune hemolytic anemia (IHA) following vaccination in children. We examined the risk of IHA in the 42 days following vaccination exposure using a self-controlled case series study design. In our population-based cohort of nearly 4.5 million children in the Vaccine Safety Datalink, we identified 55 confirmed cases of new-onset IHA from 1991 through 2000. We found no association between IHA and diphtheria-pertussis-tetanus vaccination (incidence rate ratio (IRR)=0.65, 95% CI: 0.19-2.24), hepatitis B vaccination (IRR=1.73, 95% CI: 0.59-5.01), or any vaccination (IRR=1.04, 95% CI: 0.46-2.32). PMID:19766577
Naleway, Allison L; Belongia, Edward A; Donahue, James G; Kieke, Burney A; Glanz, Jason M
Background Despite improvements on how to resuscitate exsanguinating patients, one remaining key to improve outcome is to expeditiously and reproducibly identify patients most likely to require transfusion including massive transfusion (MT). This work summarizes yet developed algorithms/scoring systems for transfusion including MT in civilian and military trauma populations. Methods A systematic search of evidence was conducted utilizing OVID/MEDLINE (1966 to present) and the ‘Medical Algorithms Project’. Results and Conclusions The models developed suggest combinations of physiologic, hemodynamic, laboratory, injury severity and demographic triggers identified on the initial evaluation of the bleeding trauma patient. Many approaches use a combination of dichotomous variables readily accessible after arrival but others rely on time-consuming calculations or complex algorithms and may have limited real-time application. Weighted and more sophisticated systems including higher numbers of variables perform superior. A common limitation to all models is their retrospective nature, and prospective validations are urgently needed. Point-of-care viscoelastic testing may be an alternative to these systems.
Maegele, Marc; Brockamp, Thomas; Nienaber, Ulrike; Probst, Christian; Schoechl, Herbert; Gorlinger, Klaus; Spinella, Philip
After prolonged intake of oral contraceptives a 28-year-old woman developed a hemolytic uremic syndrome with microangiopathic hemolytic anemia, renal failure, malignant hypertension and blindness due to papillary congestion. Renal biopsy revealed widespread arteriolar alterations with massive intimal thickening, narrowing of the lumina and fibrinoid necrosis. During treatment with anticoagulation, antihypertensives and hemodialysis the hemolysis disappeared and vision improved, but the renal failure persisted. The possible relation between oral contraceptives and hemolytic uremic syndrome is mentioned and the literature is reviewed. PMID:1215910
Blumberg, A; Studer, U; Briner, J
Erythrocyte cytosolic protein expression profiles of children with unexplained hemolytic anemia were compared with profiles of close relatives and controls by two-dimensional differential in-gel electrophoresis (2D-DIGE). The severity of anemia in the patients varied from compensated (i.e., no medical intervention required) to chronic transfusion dependence. Common characteristics of all patients included chronic elevation of reticulocyte count and a negative workup for anemia focusing on hemoglobinopathies, morphologic abnormalities that would suggest a membrane defect, immune-mediated red cell destruction, and evaluation of the most common red cell enzyme defects, glucose-6-phosphate dehydrogenase and pyruvate kinase deficiency. Based upon this initial workup and presentation during infancy or early childhood, four patients classified as hereditary nonspherocytic hemolytic anemia (HNSHA) of unknown etiology were selected for proteomic analysis. DIGE analysis of red cell cytosolic proteins clearly discriminated each anemic patient from both familial and unrelated controls, revealing both patient-specific and shared patterns of differential protein expression. Changes in expression pattern shared among the four patients were identified in several protein classes including chaperons, cytoskeletal and proteasome proteins. Elevated expression in patient samples of some proteins correlated with high reticulocyte count, likely identifying a subset of proteins that are normally lost during erythroid maturation, including proteins involved in mitochondrial metabolism and protein synthesis. Proteins identified with patient-specific decreased expression included components of the glutathione synthetic pathway, antioxidant pathways, and proteins involved in signal transduction and nucleotide metabolism. Among the more than 200 proteins identified in this study are 21 proteins not previously described as part of the erythrocyte proteome. These results demonstrate the feasibility of applying a global proteomic approach to aid characterization of red cells from patients with hereditary anemia of unknown cause, including the identification of differentially expressed proteins as potential candidates with a role in disease pathogenesis. PMID:22509282
von Löhneysen, Katharina; Scott, Thomas M; Soldau, Katrin; Xu, Xiuling; Friedman, Jeffrey S
The clinical pathologic syndrome of graft-versus-host disease (GVHD) is usually a sequela of bone marrow transplantation. This disorder occurs as a result of recognition by engrafted donor-derived lymphocytes of foreign recipient transplantation antigens. GVHD may also result from engraftment of lymphocytes from other sources, including (1) transfusion of lymphocytes containing blood components, (2) transplacental maternal fetal transfusion, and (3) passive transfer of lymphocytes in solid organ transplantation. The recipients are usually severely immunodeficient and thus incapable of rejecting the transfused lymphocytes. This syndrome may, however, also develop in immunologically competent patients receiving blood products from individuals with histocompatibility antigens not recognized as foreign. 58 refs.
Rappeport, J.M. (Yale Univ. School of Medicine, New Haven, CT (USA))
Clostridium difficile is the major identified cause of antibiotic-associated diarrhea. Metronidazole has been applied as the first-line treatment, while vancomycin has been used in recurring cases of the disease. Fecal transfusion has already long been applied as experimental therapy in the treatment of recurring C. difficile infection. The aim of fecal transfusion is normalization of the intestinal microbial flora. An only recently published extensive Finnish patient series and a randomized study have confirmed fecal transfusion as a highly efficient and safe form of treatment in recurrent C. difficile infection. PMID:24069636
Arkkila, Perttu; Mattila, Eero; Anttila, Veli-Jukka
\\u000a Abstract\\u000a Purpose To review the pathophysiology of coagulopathy in massively transfused, adult and previously hemostatically competent patients\\u000a in both elective surgical and trauma settings, and to recommend the most appropriate treatment strategies.\\u000a \\u000a \\u000a \\u000a Methods Medline was searched for articles on “massive transfusion,” “transfusion,” “trauma,” “surgery,” “coagulopathy” and “hemostatic\\u000a defects.” A group of experts reviewed the findings.\\u000a \\u000a \\u000a \\u000a Principal findings Coagulopathy will result from hemodilution, hypothermia,
Jean-François Hardy; Philippe de Moerloose; Charles Marc Samama
Othopedic surgery is a common procedure among the elderly, and patients are at risk of receiving unnecessaryblood transfusions. The goals of this project were to analyze current transfusion practices, identify opportunities for improvement, implement hospital-based quality improvement programs, and measure their impact on transfusion practices. Our aims were to decrease unnecessary transfusions and overall exposure to blood products. Data were
Anne-Marie Audet; Chester Andrzejewski; Mark Popovsky
BackgroundMultiple studies report that patients receiving red blood cell (RBC) transfusion in the intensive care unit (ICU) are more likely to experience complications. Despite these findings, surgical patients are frequently transfused for operative procedures, trauma, and burns. We hypothesized that a RBC transfusion guideline would safely decrease our use of RBC transfusions in the ICU and lower the hematocrit at
Wendy L. Wahl; Mark R. Hemmila; Paul M. Maggio; Saman Arbabi
The discovery of xenotropic murine leukemia virus-related virus (XMRV) in human tissue samples has been shown to be due to virus contamination with a recombinant murine retrovirus. However, due to the unknown pathogenicity of this novel retrovirus and its broad host range, including human cell lines, it is important to understand the modes of virus transmission and develop mitigation and management strategies to reduce the risk of human exposure and infection. XMRV transmission was evaluated by whole-blood transfusion in rhesus macaques. Monkeys were infected with XMRV to serve as donor monkeys for blood transfers at weeks 1, 2, and 3 into naïve animals. The donor and recipient monkeys were evaluated for XMRV infection by nested PCR assays with nucleotide sequence confirmation, Western blot assays for development of virus-specific antibodies, and coculture of monkey peripheral blood mononuclear cells (PBMCs) with a sensitive target cell line for virus isolation. XMRV infection was demonstrated in the virus-injected donor monkeys, but there was no evidence of virus transmission by whole-blood transfusion to naïve monkeys based upon PCR analysis of PBMCs using XMRV-specific gag and env primers, Western blot analysis of monkey plasma up to 31 to 32 weeks after transfusion, and coculture studies using monkey PBMCs from various times after transfusion. The study demonstrates the lack of XMRV transmission by whole-blood transfusion during the acute phase of infection. Furthermore, analysis of PBMC viral DNA showed extensive APOBEC-mediated G-to-A hypermutation in a donor animal at week 9, corroborating previous results using macaques and supporting the possible restriction of XMRV replication in humans by a similar mechanism.
Williams, Dhanya K.; Galvin, Teresa A.; Gao, Yamei; O'Neill, Christina; Glasner, Dustin
Background and objectives Many patients with chronic anaemia require blood transfusions as part of their treatment regimen. As a result, iron overload will inevitably develop if not adequately managed by iron chelation therapy. There are many guidelines relating to transfusion and chelation practices for patients with transfusion-dependent anaemia; however, there is a lack of information on how treatment practices differ around the world. The objective of this manuscript is to highlight key features of current transfusion and chelation management, including similarities and differences across various anaemias and between geographical regions worldwide. Materials and methods Data collected at study entry to the multicentre Evaluation of Patients’ Iron Chelation with Exjade (EPIC) study, which recruited 1,744 patients with a variety of transfusion-dependent anaemias across 23 countries from three geographic regions, were assessed. These analyses compared transfusion and chelation treatment prior to the start of study treatment, together with iron burden assessed at study entry by serum ferritin, liver iron concentration and labile plasma iron levels. Results and conclusions Data show that transfusion and iron chelation practices differ between anaemias and between geographical regions; this may be linked to availability and accessibility of transfusion and chelation therapy, patients’ compliance, physicians’ attitudes, costs and use of treatment guidelines. Approximately 60% of these transfusion-dependent patients were severely iron overloaded with a serum ferritin level over 2,500 ng/mL, indicating that the risks of iron burden may have been underestimated and current iron chelation therapy, if considered, may not have been adequate to control iron burden.
Viprakasit, Vip; Gattermann, Norbert; Lee, Jong Wook; Porter, John B.; Taher, Ali T.; Habr, Dany; Martin, Nicolas; Domokos, Gabor; Cappellini, Maria Domenica
The hemolytic activity of an extract of the mycoparasite Sepedonium chrysospermum (teleomorph Hypomyces chrysospermus) was detected and characterized. Extraction of the fungal biomass by methanol yielded a fraction in which the hemolytic activity against human red blood cells corresponded to a peptide with a molecular mass of 7,653.72 Da and an isoelectric point of approximately 5.8. The peptide was temperature resistant, and the hemolysis was only partially inhibited, even after a 30-min pre-incubation at 100°C. Its hemolytic activity was unaffected by treatment with proteolytic enzymes such as trypsin. Among the divalent cations assayed, Hg(2+) was the strongest inhibitor of hemolysis. The reducing agent, dithiothreitol, and the membrane lipid, cholesterol, demonstrated concentration-dependent inhibitory activities. Finally, hemolytic activity triggered by the peptide was analyzed by scanning electron microscopy, and a pore-forming activity was detected. PMID:22080344
Sanguineti, Elisa; Cosulich, Maria E; Salis, Annalisa; Damonte, Gianluca; Mariotti, Mauro G; Zotti, Mirca
Karenia mikimotoi (Miyake & Kominami ex Oda) Hansen & Moestrup is associated with harmful algal blooms in temperate and subtropical zones of the world. The hemolytic substances produced by K. mikimotoi are thought to cause mortality in fishes and invertebrates. We evaluated the composition of the hemolytic toxin produced by K. mikimotoi cultured in the laboratory using thin-layer chromatography. In addition, we evaluated the effect of co-occuring algae ( Prorocentrum donghaiense and Alexandrium tamarense) and the cladoceran grazer Moina mongolica on hemolytic toxin production in K. mikimotoi. The hemolytic toxins from K. mikimotoi were a mixture of 2 liposaccharides and 1 lipid. Waterborne clues from P. donghaiense and A. tamarense inhibited the growth of K. mikimotoi but increased the production of hemolytic toxins. Conversely, K. mikimotoi strongly inhibited the growth of caged P. donghaiense and A. tamarense. In addition, the ingestion of K. mikimotoi by M. mongolica induced the production of hemolytic toxins in K. mikimotoi. Taken together, our results suggest that the presence of other microalgae and grazers may be as important as environmental factors for controlling the production of hemolytic substances. K. mikimotoi secreted allelochemicals other than unstable fatty acids with hemolytic activity. The production of hemolytic toxins in dinoflagellates was not only dependent on resource availability, but also on the risk of predation. Hemolytic toxins likely play an important role as chemical deterrents secreted by K. mikimotoi.
Yang, Weidong; Zhang, Naisheng; Cui, Weimin; Xu, Yanyan; Li, Hongye; Liu, Jiesheng
We report a case of hemolytic anemia in a female patient with juvenile dermatomyositis. Rapidly progressive hemolysis developed during prednisone and azathioprine combination therapy 4 days after completing intravenous immunoglobulin (IVIG) treatment. While the blood smear revealed spherocytes and polychromasia, direct and indirect antiglobulin tests were negative. In the following case report, we propose probable IVIG-induced hemolytic anemia and explore the pathomechanisms that may account for hemolysis. PMID:23377338
Clemenz, Melanie R; McGowan Iv, Joseph Wilson; Shuler, Marshall J; Lynn, Annette W
\\u000a Pulmonary hypertension (PH) is now recognized as a complication of both chronic and acquired hemolytic anemias. The process\\u000a of hemolysis appears to be central to disease pathogenesis. Sickle cell disease (SCD), a congenital hemoglobinopathy affecting\\u000a as many as 30 million individuals worldwide, is the best characterized hemolytic anemia associated with PH. Multiple clinical\\u000a studies have demonstrated a 10–30% prevalence of
Elizabeth S. Klings; Mark T. Gladwin
We have previously found that the methanol extract prepared from Heterocapsa circularisquama, a harmful red rid dinoflagellate, showed light-dependent hemolytic activity toward rabbit erythrocytes. Interestingly, the cytotoxicity of the extract against HeLa cells was also strictly light-dependent, and no significant toxic effect was observed even at very high concentration in the dark. In this study, the hemolytic agents present in
Yousuke Miyazaki; Takuji Nakashima; Takashi Iwashita; Tsuyoshi Fujita; Kenichi Yamaguchi; Tatsuya Oda
Cold agglutinin induced autoimmune hemolytic anemia is uncommonly associated with leukemia and lymphomas. We present a case of a young Mexican female presenting with a cold agglutinin hemolytic anemia with expression of a rare Pr antigen specificity and an aggressive NK-cell leukemia. Our patient had a rapid fatal course. To our knowledge this is the first reported case of such an association. PMID:17301976
Skorupa, Amy; Chaudhary, Uzair B; Lazarchick, John
Transfusion of syngeneic marrow into normal, nonirradiated recipients results only in minimal proliferation of donor cells. However, irradiated recipients, restored to hematologic normalcy by an initial marrow transfusion, subsequently sustain proliferation which replaces approximately 10% of endogenous marrow after a single transfusion of 4 x 10/sup 7/ marrow cells of the same strain as the host. Cells from histoincompatible donors proliferate only rarely or minimally in the marrows of these irradiated, but hematologically normal recipients without reirradiation. Syngeneic male donor cells proliferate in irradiated and restored female mice, while female donor cells fail to proliferate in the marrow of syngeneic male recipients. A possible explanation is that transfused female cells respond immunologically to the abundant H-Y antigen in the male environment and are eliminated as a result.
Brecher, G. (Univ. of California, Berkeley); Lawce, H.; Tjio, J.H.
...availability of blood and blood products. (b) Provision of testing. The facility must provide prompt ABO grouping, D(Rho) typing, unexpected antibody detection, compatibility testing, and laboratory investigation of transfusion reactions on a...
... Hindi (??????) Japanese (???) Korean (???) Portuguese (português) Russian (???????) Somali (af Soomaali) Spanish (español) Tagalog ( ... PDF Health Information Translations Return to top Portuguese (português) Receiving Blood Transfusions Transfusões de sangue - português (Portuguese) ...
Text Version... ldentification System for Transfusion Management is a software application that is designed to be used in a client server environment, along with ... More results from www.fda.gov/downloads/biologicsbloodvaccines/bloodbloodproducts
A Positive Donor Recipient Identification System for a Transfusion Service has been developed. Donor and recipient identification are established by a human and machine readable number. The Recipient ID System utilizes the standard hospital ID bracelet an...
R. Chambers M. Rubin G. Sandler T. Macnamara C. Rath
This paper explores the scriptural and theological reasons given by Jehovah's Witnesses (JWs) to refuse blood transfusions. Julian Savulescu and Richard W Momeyer argue that informed consent should be based on rational beliefs and that the refusal of blood transfusions by JWs is irrational, but after examining the reasons given by JWs, I challenge the claim that JW beliefs are irrational. I also question whether we should give up the traditional notion of informed consent. PMID:22790086
Bock, Gregory L
Background and objectives: Although well-described for patients who require dialysis, information on transfusion burden related to anemia in the nondialysis patient population with chronic kidney disease (CKD) is lacking. Design, settings, participants, & measurements: A retrospective study was conducted of patients with CKD and chronic anemia from 2002 through 2007 in the Veterans Administration Healthcare System. Included patients had stage 3 CKD or higher and anemia (one or more hemoglobin [Hb] levels <11 g/dl or received anemia therapy [erythropoiesis-stimulating agents [ESAs], iron, or both]). The outcome of interest was transfusion events, which was evaluated in relation to the absolute Hb level and changes in Hb levels overall and according to the type of treatment received (no treatment, iron therapy, ESA therapy, or ESA and iron therapy) concurrent with each Hb measurement. Results: Among 97,636 patients with CKD and anemia, we observed 68,556 transfusion events (61 events per 100 person-years), 86.6% of which occurred in inpatient settings. At all Hb levels, transfusion events were highest during periods of no treatment and increased with declining Hb levels. Between an Hb of 10.0 and 10.9 g/dl, the transfusion rate was 2.0% for those who received an ESA, iron, or both and 22% for those who received no treatment; at an Hb level of 7.0 to 7.9 g/dl, the transfusion rate was 10 to 12% for treated and 58% for untreated patients. Low absolute Hb levels but not Hb changes was most predictive of a transfusion even after adjustment for patient case mix. Conclusions: Transfusions are still used to treat anemia in patients who have CKD and do not require dialysis, although they occur considerably less frequently in patients who receive other available anemia therapies.
Bradbury, Brian D.; Fonda, Jennifer R.; Gaziano, J. Michael; Gagnon, David R.
The outbreak of Shiga toxin producing E.coli O104:H4 in northern Germany in 2011 was one of the largest worldwide and involved mainly adults. Post-diarrheal hemolytic uremic syndrome (HUS) occurred in 22% of STEC positive patients. This study's aim was to assess risk factors for HUS in STEC-infected patients and to develop a score from routine hospital parameters to estimate patient risks for developing HUS. In a cohort analysis, adult patients with STEC infection were included in five participating hospitals in northern Germany between May and July 2011. Clinical data were obtained from questionnaires and medical records, laboratory data were extracted from hospitals' electronic data systems. HUS was defined as thrombocytopenia, hemolytic anemia and acute renal dysfunction. Random forests and multivariate logistic regression were used to identify risk factors for HUS and develop a score using the estimated coefficients as weights. Among 259 adults with STEC infection, vomiting (OR 3.48,95%CI 1.88-6.53), visible blood in stools (OR 3.91,95%CI1.20-16.01), age above 75 years (OR 3.27, 95%CI 1.12-9.70) and elevated leukocyte counts (OR 1.20, 95%CI 1.10-1.31, per 1000 cells/mm(3)) were identified as independent risk factors for HUS. A score using these variables has an area under the ROC curve of 0.74 (95%CI 0.68-0.80). Vomiting, visible blood in stools, higher leukocyte counts, and higher age indicate increased risk for developing HUS. A score using these variables might help to identify high risk patients who potentially benefit from aggressive pre-emptive treatment to prevent or mitigate the devastating consequences of HUS. PMID:23533606
Zoufaly, Alexander; Cramer, Jakob P; Vettorazzi, Eik; Sayk, Friedhelm; Bremer, Jan P; Koop, Irmtraut; de Weerth, Andreas; Schmiedel, Stefan; Jordan, Sabine; Fraedrich, Katharina; Asselborn, Niels H; Nitschke, Martin; Neumann-Grutzeck, Christine; Magnus, Tim; Rüther, Christoph; Fellermann, Klaus; Stahl, Rolf K; Wegscheider, Karl; Lohse, Ansgar W
In 1990, the Pediatric Hemotherapy Committee of the American Association of Blood Banks developed and distributed a questionnaire addressing neonatal blood transfusion practices. The same questionnaire was subsequently sent to Canadian university-affiliated hospitals (n = 92). This report describes the results of the Canadian survey. Seventy-two percent (n = 66) of institutions contacted responded. Of these 42% (n = 28) had sufficient experience with neonatal transfusions and provided sufficient data for analysis. Although the majority of stated practices did follow published guidelines, several areas of variability and/or suboptimal practices were identified. With respect to component selection and preparation, suboptimal practices included excessive pretransfusion testing, unnecessary routine washing of RBC concentrates for small-volume transfusions, routine volume reduction of platelet concentrates and the use of suboptimal granulocyte preparations. With respect to transfusion practices, a disturbingly high percentage of respondents indicated that frozen plasma would be given in situations generally considered inappropriate. There was a great deal of variability in the provision of blood components at low risk for CMV, in the use of gamma irradiation and in the platelet count used for prophylactic platelet transfusions. The data collected in this survey provide information concerning practices that require improvement, identify areas where further research is desirable and provide a basis for comparison with current and future neonatal blood transfusion practices. PMID:10174295
Hume, H; Blanchette, V; Strauss, R G; Levy, G J
To determine the role of polymorphonuclear (PMN) leukocyte transfusions in neonates with sepsis, 23 consecutive newborns were prospectively randomly selected during an 18-month period in a treatment plan to receive polymorphonuclear leukocyte transfusions with supportive care or supportive care alone. Thirteen neonates received transfusions every 12 hours for a total of five transfusions. Each transfusion consisting of 15 mL/kg of polymorphonuclear leukocytes was subjected to 1,500 rads of radiation. The polymorphonuclear leukocytes were obtained by continuous-flow centrifugation leukapheresis and contained 0.5 to 1.0 X 10(9) granulocytes per 15 mL with less than 10% lymphocytes. Positive findings on blood cultures were obtained in 14/23 patients and seven were randomly selected for each treatment group. Absolute granulocyte counts were less than 1,500/microL in 13 patients but tibial bone marrow examinations revealed that the neutrophil supply pool was depleted in only three patients. The survival was significantly greater in the treatment group compared with the group that did not receive transfusions.
Cairo, M.S.; Rucker, R.; Bennetts, G.A.; Hicks, D.; Worcester, C.; Amlie, R.; Johnson, S.; Katz, J.
Objective Preoperative over-ordering of blood is common and leads to the wastage of blood bank resources. The preoperative blood ordering and transfusion practices for common elective general surgical procedures were evaluated in our university hospital to formulate a maximum surgical blood order schedule (MSBOS) for those procedures where a cross-match appears necessary. Methods We evaluated blood ordering practices retrospectively in all elective general surgical procedures in our institution over a 6-month period. Cross-match-to-transfusion ratios (C:T) were calculated and compared to current trust and the British Society of Haematology (BSH) guidelines. The adjusted C:T ratio was also calculated and was defined as the C:T ratio when only cross-matched blood used intraoperatively was included in the calculation. Results 541 patients were identified during the 6-month period. There were 314 minor and 227 major surgeries carried out. 99.6% (n = 226) of the patients who underwent major surgery and 95.5% (n = 300) of the patients having minor surgery had at least a group and save (G and S) test preoperatively. A total of 507 units of blood were cross-matched and 238 units were used. The overall C:T ratio was therefore 2.1:1, which corresponds to a 46.9% red cell usage. There was considerable variation in the C:T ratio, depending on the type of surgery performed. The adjusted C:T ratio varied between 3.75 and 37. Conclusions Compliance with transfusion policies is poor and over-ordering of blood products commonplace. Implementation of the updated recommended MSBOS and introduction of G and S for eligible surgical procedures is a safe, effective and cost-effective method to prevent preoperative over-ordering of blood in elective general surgery. Savings of GBP 8,596.00 per annum are achievable with the incorporation of updated evidence-based guidelines in our university hospital.
Hall, Thomas C.; Pattenden, Clare; Hollobone, Chloe; Pollard, Cristina; Dennison, Ashley R.
Transfusion and hematologic variables after fibrinogen or platelet transfusion in valve replacement surgery: preliminary data of purified lyophilized human fibrinogen concentrate versus conventional transfusion.
BACKGROUND: Platelet (PLT) and plasma transfusion remain the mainstay hemostatic therapy for perioperative bleeding. Several studies have indicated that acquired fibrinogen (FIB) deficiency can be the primary cause of bleeding after cardiac surgery. The aim of this study was to compare hematologic and transfusion profiles between the first-line FIB replacement and PLT transfusion in post-cardiac surgical bleeding. STUDY DESIGN AND METHODS: In this prospective, randomized, open-label study, 20 adult patients who underwent valve replacement or repair and fulfilled preset visual bleeding scale were randomized to 4?g of FIB or 1 unit of apheresis PLTs. Primary endpoints included hemostatic condition in the surgical field and 24-hour hemostatic product usage. Hematologic data, clinical outcome, and safety data were collected up to the 28th day postoperative visit. RESULTS: In patients who received the first-line FIB concentrate (n?=?10), the visual bleeding scale improved after intervention, and the incidence of PLT transfusion and total plasma donor exposure were lower compared to the PLT group (n?=?10). Postintervention FIB level was statistically higher (209?mg/dL vs. 165?mg/dL) in the FIB group than in the PLT group, but PLT count and prothrombin were lower. There were no statistical differences in the postoperative blood loss and red blood cell transfusion between two groups. CONCLUSIONS: Our preliminary data indicate that the primary FIB replacement may potentially reduce the incidence of PLT transfusion and the number of donor exposures. Plasma FIB level of 200?mg/dL is attainable with a single dose of 4?g, and this level seems to mitigate bleeding despite moderately decreased thrombin generation. PMID:23718572
Tanaka, Kenichi A; Egan, Katherine; Szlam, Fania; Ogawa, Satoru; Roback, John D; Sreeram, Gautam; Guyton, Robert A; Chen, Edward P
Attempts to detect infectivity in the blood of humans and animals affected with transmissible spongiform encephalopathies (TSEs or prion diseases) have often been inconclusive because of the limitations of cross-species bioassays and the small volumes of blood that can be injected by the intracerebral route. A model has been developed for the experimental study of TSE transmission by blood transfusion using sheep experimentally infected with bovine spongiform encephalopathy (BSE) or natural scrapie as donors and susceptible scrapie-free sheep as recipients. Donors and recipients of the same species greatly increase the sensitivity of the bioassay and in sheep large volumes of blood can be injected by the intravenous (i.v.) route. Transmission of BSE to a single animal using this approach was reported recently. This study confirms this result with a second transmission of BSE and four new cases of transmission of natural scrapie. Positive transmissions occurred with blood taken at pre-clinical and clinical stages of infection. Initial studies indicate that following such infection by the i.v. route, deposition of the abnormal prion protein isoform, PrP(Sc), in peripheral tissues may be much more limited than is seen following oral infection. These results confirm the risks of TSE infection via blood products and suggest that the measures taken to restrict the use of blood in the UK have been fully justified. PMID:12388826
Hunter, Nora; Foster, James; Chong, Angela; McCutcheon, Sandra; Parnham, David; Eaton, Samantha; MacKenzie, Calum; Houston, Fiona
Hemolytic-uremic syndrome (HUS) is a systemic disease characterized by microvascular endothelial damage, mainly in the gastrointestinal tract and the kidneys. A major cause of HUS is Shiga toxigenic Escherichia coli (STEC) infection. In addition to Shiga toxin, additional STEC virulence factors may contribute to HUS. One is the newly discovered subtilase cytotoxin (SubAB), which is highly toxic to eukaryotic cells, and when injected intraperitoneally into mice causes pathology resembling that associated with human HUS. Recent data show that SubAB exhibits a strong preference for glycans terminating in ?2-3-linked N-glycolylneuraminic acid (Neu5Gc), a sialic acid that humans are unable to synthesize, because we genetically lack the necessary enzyme. However, Neu5Gc can still be found on human cells due to metabolic incorporation from the diet. Dietary incorporation happens to be highest in human endothelium and to a lesser extent in the intestinal epithelium, the two affected cell types in STEC-induced HUS. Mammalian-derived foods such as red meat and dairy products appear to be the primary source of dietary Neu5Gc. Ironically, these are also common sources of STEC contamination. Taken together, these findings suggest a ‘two-hit’ process in the pathogenesis of human SubAB-induced disease. First, humans eat Neu5Gc-rich food, leading to incorporation of Neu5Gc on the surfaces of endothelial and intestinal cells. Second, when exposed to a SubAB-producing STEC strain, the toxin produced would be able to bind to the intestinal epithelial cells, perhaps causing acute gastrointestinal symptoms, and eventually damaging endothelial cells in other organs like the kidney, thereby causing HUS.
Lofling, Jonas C.; Paton, Adrienne W.; Varki, Nissi M.; Paton, James C.; Varki, Ajit
The purpose of this study was to compare the bacteriologic and clinical efficacy of oral penicillin versus amoxicillin as first-line therapy for group A beta-hemolytic streptococcal (GABHS) tonsillopharyngitis. The prospective observational study was conducted over 18 months (January 2000-June 2001). Children enrolled had acute onset of symptoms and signs and a laboratory-documented GABHS tonsillopharyngitis illness. Follow-up examination and laboratory testing occurred 10 +/- 4 days following completion of treatment. In total, 389 patients were enrolled (intent-to-treat group): 195 received penicillin V and 194 received amoxicillin. Fifty-six of the penicillin-treated and 57 amoxicillin-treated patients refused to take the drug, or were noncompliant, or did not return for the follow-up visit, leaving 276 patients in the per-protocol group: 139 penicillin-treated and 137 amoxicillin-treated. Bacteriologic cure for amoxicillin-treated children occurred in 76% versus 64% in the penicillin-treated children (p = 0.04). The clinical cure rate for amoxicillin-treated children was 84% compared to 73% in the penicillin-treated children (p = 0.03). Since treatment allocation was not randomized, logistic regression analysis was used to adjust for treatment group differences. The odds ratio (OR) estimate for cure for patients in the amoxicillin versus penicillin V treatment group remained significant (OR = 1.84, 95% confidence interval 1.02-3.29); the same was true for dinical cure (OR = 1.99, 95% CI = 1.02-3.87). Amoxicillin may be superior to penicillin for bacteriologic and clinical cure of GABHS tonsillopharyngitis. PMID:12739920
Curtin-Wirt, Correne; Casey, Janet R; Murray, Patrick C; Cleary, Carolyn T; Hoeger, William J; Marsocci, Steven M; Murphy, Marie Lynd; Francis, Anne B; Pichichero, Michael E
The cases of lymphoma accompanied or preceded by Coombs' test positive autoimmune hemolytic anemia (AIHA) have been reported. However, Coombs' test negative AIHA prior to the diagnosis of lymphoma was rarely described. Herein, this article reports a case of non-Hodgkin's lymphoma (NHL) preceded about 1.5 years by Coombs test negative AIHA. A woman aged 69 was diagnosed with HA based on the history and laboratory tests. Further studies revealed that this patient was negative with Coombs' test for IgG, IgM, IgA and C3. After all possible causes of HA, especially malignancies were ruled out, the patient was diagnosed with Coombs' test negative AIHA and treated with prednisolone. The patient responded well initially to steroid treatment. Two recurrences of acute HA were presented at time of 10 months post steroid cessation, and immediately after an attempt to withdraw steroid, respectively, but the hemolysis was effectively controlled by reinstitution of prednisolone. At third recurrence, however, the patient was no longer responding to steroid, and was found with cervical lymphadenopathy. Coombs' test for IgG, IgM, IgA and C3 remained negative. B cell NHL was diagnosed by pathology. After receiving 6 cycles of CHOP chemotherapy, the patient was lymphoma free, but the hemolysis was not improved, however, which was effectively controlled by the following low dose-rituximab (RTX) therapy. The patient was still kept in a remission of lymphoma free of anemia. In conclusion, this report presented a very rare case of NHL with Coombs' test negative AIHA as initial major clinical manifestation. PMID:22391174
Wan, Sui-Gui; Lin, Yang; Xia, Chang-Qing; Zhao, Hong; Xu, Juan
Over the past few decades, transfusion medicine and haemotherapy have evolved into complex medical disciplines comprising a broad field of subspecialties such as immunohaematology, blood component production, haemapheresis and haemostaseology. Transfusion medicine is thus an important qualification at the interfaces of analytical laboratory medicine, pharmaceutical production and clinical disciplines such as internal medicine, anaesthesiology or surgery. Physicians specialising in transfusion
M. M. Mueller; E. Seifried
DEAP-HUS (deficiency of CFHR plasma proteins and factor H [FH] autoantibody positive hemolytic uremic syndrome [HUS]) is a new form of HUS characterized by a deletion of genes coding for FH-related proteins and the presence of autoantibodies directed to FH. These disease-associated autoantibodies inhibit FH (CFH) surface binding functions, which results in a defective regulation of the alternative pathway and damage of endothelial cells. Here we describe two representative patients with DEAP-HUS who both developed end-stage renal failure with the background of homozygous deletion of CFHR1 and CFHR3 genes and the presence of FH autoantibodies. Based on the retrospective diagnosis of DEAP-HUS 2 to 12 months after the initial clinical presentation, subsequent immunosuppressive therapy was initiated. The autoantibody titers decreased, and the complement status of the patients improved, as indicated by increased C3 levels. Thus early diagnosis of DEAP-HUS and immunosuppressive treatments are important factors to treat this particular type of HUS. PMID:20865639
Skerka, Christine; Zipfel, Peter F; Müller, Dominik; Micklisch, Sven; Riedl, Magdalena; Zimmerhackl, Lothar-Bernd; Hofer, Johannes
Eculizumab (anti-C5) has been sporadically reported as an efficient therapy for atypical hemolytic uremic syndrome (aHUS). However, the lack of series precludes any firm conclusion about the optimal use of anti-C5 for preventing or treating aHUS posttransplant aHUS recurrence. We thoroughly studied 22 renal transplant recipients with aHUS who received off-label therapy with anti-C5, including 12 cases, which have not been reported yet. Nine patients, all carrying a complement genetic abnormality associated with a high risk of aHUS recurrence, received prophylactic anti-C5 therapy to prevent posttransplant recurrence. Eight of them had a successful recurrence-free posttransplant course and achieved a satisfactory graft function, while the remaining patient experienced early arterial thrombosis of the graft. Thirteen renal transplant recipients were given anti-C5 for posttransplant aHUS recurrence. A complete reversal of aHUS activity was obtained in all of them. Importantly, the delay of anti-C5 initiation after the onset of the aHUS episode inversely correlated with the degree of renal function improvement. Three patients in whom anti-C5 was subsequently stopped experienced a relapse. Altogether these data suggest that long-term eculizumab is highly effective for preventing and treating posttransplant aHUS recurrence. Our study also indicates that anti-C5 should be promptly started if a recurrence occurs. PMID:22958221
Zuber, J; Le Quintrec, M; Krid, S; Bertoye, C; Gueutin, V; Lahoche, A; Heyne, N; Ardissino, G; Chatelet, V; Noël, L-H; Hourmant, M; Niaudet, P; Frémeaux-Bacchi, V; Rondeau, E; Legendre, C; Loirat, C
Atypical hemolytic uremic syndrome (aHUS) is a disease caused by pathologies in the alternative complement system. The prevalence of aHUS is 10% of all aHUS cases. The subgroup of aHUS designated as DEAP (DEficiency of CFHR Proteins and CFH Autoantibody Positive)-HUS because of autoantibody to complement factor H (CFH) and CFH-related protein deficiency is seen very rarely, and the prevalence is 6% of all aHUS cases in the literature. We present here a female patient with DEAP-HUS. A 7.5-year-old girl with recurrent attacks of HUS had low C3 level. We initiated plasmapheresis treatment. After further analysis of the complement system, the result was compatible with DEAP-HUS, so we initiated immunosuppressive treatment. There were also family members with deficiency of CFHR-1 and CFHR-3, but they had no CFH autoantibody and no symptoms of HUS. In atypical cases of HUS, we should investigate complement status, especially for factor H autoantibody, for which treatment options differ from those of the other types of aHUS. PMID:23692839
Uslu-Gökceo?lu, Arife; Do?an, Cagla Serpil; Comak, Elif; Koyun, Mustafa; Akman, Sema
Complement dysregulation leads to atypical hemolytic uremic syndrome (aHUS), while ADAMTS13 deficiency causes thrombotic thrombocytopenic purpura. We investigated whether genetic variations in the ADAMTS13 gene partially explain the reduced activity known to occur in some patients with aHUS. We measured complement activity and ADAMTS13 function, and completed mutation screening of multiple complement genes and ADAMTS13 in a large cohort of aHUS patients. In over 50% of patients we identified complement gene mutations. Surprisingly, 80% of patients also carried at least 1 nonsynonymous change in ADAMTS13, and in 38% of patients, multiple ADAMTS13 variations were found. Six of the 9 amino acid substitutions in ADAMTS13 were common single nucleotide polymorphisms; however, 3 variants-A747V, V832M, and R1096H- were rare, with minor allele frequencies of 0.0094%, 0.5%, and 0.32%, respectively. Reduced complement and ADAMTS13 activity (<60% of normal activity) were found in over 60% and 50% of patients, respectively. We concluded that partial ADAMTS13 deficiency is a common finding in aHUS patients and that genetic screening and functional tests of ADAMTS13 should be considered in these patients. PMID:23847193
Feng, Shuju; Eyler, Stephen J; Zhang, Yuzhou; Maga, Tara; Nester, Carla M; Kroll, Michael H; Smith, Richard J; Afshar-Kharghan, Vahid
Atypical hemolytic uremic syndrome (aHUS), is mainly present in children, who have high risks of end-stage kidney disease (ESKD), post-transplant recurrence and death. aHUS is linked to defective regulation of the complement alternative pathway (AP), with a prominent cause being mutation/inhibition of the negative regulator complement factor H (CFH). CFH function can be restored via infusion of fresh frozen plasma (FFP), a treatment that was effective for several years in a patient heterozygous for a cfh mutation, before the patient progressed to ESKD. While on dialysis, FFP was replaced with eculizumab, which blocks C5 cleavage and thus halts progression of the terminal complement pathway. Patient plasma samples collected during FFP and eculizumab treatment phases were assessed for AP activity (via erythrocyte lysis assays) and for overall complement activity (via ELISA-based screen). Assay results indicated that FFP partially restored AP regulation, an observation supported by in vitro modeling of FFP treatment using purified CFH, while eculizumab completely blocked complement activity. The same approach was used to model in vitro a potential aHUS treatment approach based on blocking the AP effector properdin (complement factor P; CFP) with an anti-properdin antibody. These results provide insights into the efficacy of aHUS treatment and highlight the usefulness of in vitro assays in monitoring and predicting therapeutic responses and testing new treatment possibilities. PMID:23220071
Heinen, Stefan; Pluthero, Fred G; van Eimeren, Viola F; Quaggin, Susan E; Licht, Christoph
AIM: To clarify the incidence of congenital hemolytic anemias (CHA) in young cholelithiasis patients and to determine a possible screening test based on the results. METHODS: Young cholelithiasis patients (< 35 years) were invited to our outpatient clinic. Participants were asked for comorbidities and family history. The number of gallstones were recorded. Blood samples were obtained to perform a complete blood count, standard Wright-Giemsa staining, reticulocyte count, hemoglobin (Hb) electrophoresis, serum lactate dehydrogenase and bilirubin levels, and lipid profile. RESULTS: Of 3226 cholecystectomy patients, 199 were under 35 years, and 190 with no diagnosis of CHA were invited to take part in the study. Fifty three patients consented to the study. The median age was 29 years (range, 17-35 years), 5 were male and 48 were female. Twelve patients (22.6%) were diagnosed as thalassemia trait and/or ?ron-deficiency anemia. Hb levels were significantly lower (P = 0.046), and mean corpuscular volume (MCV) and hematocrit levels were slightly lower (P = 0.072 and 0.082, respectively) than normal. There was also a significantly lower number of gallstones with the diagnosis (P = 0.007). CONCLUSION: In endemic regions, for young cholelithiasis patients (age under 35) with 2-5 gallstones, the clinician/surgeon should pay attention to MCV and Hb levels as indicative of CHA.
Ezer, Ali; Torer, Nurkan; Nursal, Tarik Zafer; Kizilkilic, Ebru; Caliskan, Kenan; Colakoglu, Tamer; Moray, Gokhan
Autoimmune hemolytic anemia (AIHA) is an uncommon autoantibody-mediated immune disorder that affects both children and adults. The diagnosis of AIHA relies mainly on the direct antiglobulin test, which is a highly sensitive and relatively specific test. The classification of AIHA is based on the pattern of the direct antiglobulin test and on the immunochemical properties of the autoantibody (warm or cold type), but also on the presence or absence of an underlying condition or disease (secondary vs primary AIHAs) that may have an impact on treatment and outcome. The distinction between AIHAs due to warm antibody (wAIHA) and AIHAs due to cold antibody is a crucial step of the diagnostic procedure as it influences the therapeutic strategy. Whereas corticosteroids are the cornerstone of treatment in wAIHA, they have no or little efficacy in cold AIHA. In wAIHA that is refractory or dependent to corticosteroids, splenectomy and rituximab are both good alternatives and the benefit?risk ratio of each option must be discussed on an individual basis. In chronic agglutinin disease, the most common variety of cold AIHA in adults, beyond supportive measures, rituximab given either alone or in combination with chemotherapy may be helpful. In this article, the classification of AIHA and the recent progress in therapeutics are discussed. PMID:22077525
We used gel centrifugation tests (GCTs) to analyze the relationship between the diagnosis and immunohematology tests used for autoimmune hemolytic anemia (AIHA). The study included 588 samples positive for the direct antiglobulin test (DAT). Of these, 52 were from patients diagnosed with AIHA. Immunoglobulin (Ig) class, IgG1, IgG3, and complement were measured. DAT strength had the strongest correlation with AIHA diagnosis (odds ratio [OR], 23), followed by anti-IgG titer 300 (OR, 8.4), anti-IgG titer 1,000 (OR, 10.5), and C3d agglutination strength (OR, 1.7). Decision tree analysis revealed that DAT strength and anti-IgG titer higher than 100 were the best predictors of AIHA. Multidimensional scanning analysis found a high grade of similarity among DAT strength, anti-IgG titer, and IgG strength in the AIHA samples. This observation was not detected in DAT-positive samples from patients without AIHA. DAT strength remained the best diagnostic indicator for AIHA and had the strongest association with AIHA compared with other commercially available immunohematology tests. The other tests, despite good correlation with AIHA diagnosis, did not add useful information. PMID:23525616
Lai, Marco; Leone, Giuseppe; Landolfi, Raffaele
We conducted prospective surveillance of childhood hemolytic uremic syndrome (HUS) from 1997 to 2001 to describe disease incidence and clinical, epidemiologic and microbiologic characteristics. We compared our findings, where possible, with those of a previous study conducted from 1985 to 1988. The average annual incidence of HUS for the United Kingdom and Ireland (0.71/100,000) was unchanged from 1985 to 1988. The overall early mortality had halved, but the reduction in mortality was almost entirely accounted for by improved outcome in patients with diarrhea-associated HUS. The principal infective cause of diarrhea-associated HUS was Shiga toxin–producing Escherichia coli O157 (STEC O157), although in the 1997–2001 survey STEC O157 phage type (PT) 21/28 had replaced STEC O157 PT2 as the predominant PT. The risk of developing diarrhea-associated HUS was significantly higher in children infected with STEC O157 PT 2 and PT 21/28 compared with other PTs. Hypertension as a complication of HUS was greatly reduced in patients with diarrhea-associated HUS.
Lynn, Richard M.; O'Brien, Sarah J.; Taylor, C. Mark; Adak, Goutam K.; Chart, Henrik; Cheasty, Tom; Coia, John E.; Gillespie, Iain A.; Locking, Mary E.; Reilly, William J.; Smith, Henry R.; Waters, Aoife; Willshaw, Geraldine A.
Background.?Postdiarrheal hemolytic uremic syndrome (HUS) is the most common cause of acute kidney failure among US children. The Foodborne Diseases Active Surveillance Network (FoodNet) conducts population-based surveillance of pediatric HUS to measure the incidence of disease and to validate surveillance trends in associated Shiga toxin–producing Escherichia coli (STEC) O157 infection. Methods.?We report the incidence of pediatric HUS, which is defined as HUS in children <18 years. We compare the results from provider-based surveillance and hospital discharge data review and examine the impact of different case definitions on the findings of the surveillance system. Results.?During 2000–2007, 627 pediatric HUS cases were reported. Fifty-two percent of cases were classified as confirmed (diarrhea, anemia, microangiopathic changes, low platelet count, and acute renal impairment). The average annual crude incidence rate for all reported cases of pediatric HUS was 0.78 per 100 000 children <18 years. Regardless of the case definition used, the year-to-year pattern of incidence appeared similar. More cases were captured by provider-based surveillance (76%) than by hospital discharge data review (68%); only 49% were identified by both methods. Conclusions.?The overall incidence of pediatric HUS was affected by key characteristics of the surveillance system, including the method of ascertainment and the case definitions. However, year-to-year patterns were similar for all methods examined, suggesting that several approaches to HUS surveillance can be used to track trends.
Ong, Kanyin L.; Apostal, Mirasol; Comstock, Nicole; Hurd, Sharon; Webb, Tameka Hayes; Mickelson, Stephanie; Scheftel, Joni; Smith, Glenda; Shiferaw, Beletshachew; Boothe, Effie
Stonustoxin (SNTX) is a pore-forming cytolytic lethal factor, isolated from the venom of the stonefish Synanceja horrida, that has potent hemolytic activity. The role of tryptophan residues in the hemolytic activity of SNTX was investigated. Oxidation of tryptophan residues of SNTX with N-bromosuccinimide (NBS) resulted in loss of hemolytic activity. Binding of 8-anilino-1-naphthalenesulphonate (ANS) to SNTX resulted in occlusion of
Wen Shan Yew; Hoon Eng Khoo
Stroke is a devastating complication of sickle cell anemia (SCA) with high recurrence if untreated. Chronic transfusions reduce recurrent strokes but have associated morbidities including iron overload. Stroke With Transfusions Changing to Hydroxyurea (SWiTCH) was a multicenter phase 3 randomized trial comparing standard treatment (transfusions/chelation) to alternative treatment (hydroxyurea/phlebotomy) for children with SCA, stroke, and iron overload. SWiTCH was a noninferiority trial with a composite primary end point, allowing an increased stroke risk but requiring superiority for removing iron. Subjects on standard treatment received monthly transfusions plus daily deferasirox iron chelation. Subjects on alternative treatment received hydroxyurea plus overlap transfusions during dose escalation to maximum tolerated dose (MTD), followed by monthly phlebotomy. Subjects on standard treatment (N = 66) maintained 30% sickle hemoglobin (HbS) and tolerated deferasirox at 28.2 ± 6.0 mg/kg/d. Subjects on alternative treatment (N = 67) initiated hydroxyurea and 60 (90%) reached MTD at 26.2 ± 4.9 mg/kg/d with 29.1% ± 6.7% fetal hemoglobin (HbF). Adjudication documented no strokes on transfusions/chelation but 7 (10%) on hydroxyurea/phlebotomy, still within the noninferiority stroke margin. The National Heart, Lung, and Blood Institute closed SWiTCH after interim analysis revealed equivalent liver iron content, indicating futility for the composite primary end point. Transfusions and chelation remain a better way to manage children with SCA, stroke, and iron overload. This clinical trial was registered at ClinicalTrials.gov NCT00122980.
Helms, Ronald W.
Introduction Anemia is one of the most common medical complications to be encountered in critically ill patients. Based on the results of clinical trials, transfusion practices across the world have generally become more restrictive. However, because reduced oxygen delivery contributes to 'secondary' cerebral injury, anemia may not be as well tolerated among neurocritical care patients. Methods The first portion of this paper is a narrative review of the physiologic implications of anemia, hemodilution, and transfusion in the setting of brain-injury and stroke. The second portion is a systematic review to identify studies assessing the association between anemia or the use of red blood cell transfusions and relevant clinical outcomes in various neurocritical care populations. Results There have been no randomized controlled trials that have adequately assessed optimal transfusion thresholds specifically among brain-injured patients. The importance of ischemia and the implications of anemia are not necessarily the same for all neurocritical care conditions. Nevertheless, there exists an extensive body of experimental work, as well as human observational and physiologic studies, which have advanced knowledge in this area and provide some guidance to clinicians. Lower hemoglobin concentrations are consistently associated with worse physiologic parameters and clinical outcomes; however, this relationship may not be altered by more aggressive use of red blood cell transfusions. Conclusions Although hemoglobin concentrations as low as 7 g/dl are well tolerated in most critical care patients, such a severe degree of anemia could be harmful in brain-injured patients. Randomized controlled trials of different transfusion thresholds, specifically in neurocritical care settings, are required. The impact of the duration of blood storage on the neurologic implications of transfusion also requires further investigation.
Kramer, Andreas H; Zygun, David A
Background: Blood transfusion therapy (BTT), which represents transplantation of living cells, poses several risks. Although BTT is necessary for trauma victims with hemorrhagic shock, it may be futile for patients with blunt traumatic cardiopulmonary arrest (BT-CPA). Materials and Methods: We retrospectively examined the medical records of consecutive patients with T-CPA. The study period was divided into two periods: The first from 1995-1998, when we used packed red cells (PRC) regardless of the return of spontaneous circulation (ROSC), and the second from 1999-2004, when we did not use PRC before ROSC. The rates of ROSC, admission to the ICU, and survival-to-discharge were compared between these two periods. Results: We studied the records of 464 patients with BT-CPA (175 in the first period and 289 in the second period). Although the rates of ROSC and admission to the ICU were statistically higher in the first period, there was no statistical difference in the rate of survival-to-discharge between these two periods. In the first period, the rate of ROSC was statistically higher in the non-BTT group than the BTT group. However, for cases in which ROSC was performed and was successful, there were no statistical differences in the rate of admission and survival-to-discharge between the first and second group, and between the BTT and non-BTT group. Conclusion: Our retrospective consecutive study shows the possibility that BTT before ROSC for BT-CPA and a treatment strategy that includes this treatment improves the success rate of ROSC, but not the survival rate. BTT is thought to be futile as a treatment for BT-CPA before ROSC.
Moriwaki, Yoshihiro; Sugiyama, Mitsugi; Tahara, Yoshio; Iwashita, Masayuki; Kosuge, Takayuki; Toyoda, Hiroshi; Arata, Shinju; Suzuki, Noriyuki
We present a case of successful living donor liver transplantation (LDLT) for liver cirrhosis caused by hepatitis B virus with severe autoimmune hemolytic anemia (AIHA) using an ABO-incompatible (ABOi) graft. The patient was a 47-year-old woman who had a history of ruptured esophageal varices, accumulation of intractable ascites, frequent hepatic encephalopathy and severe anemia, with a hemoglobin value of approximately 3 g/dL due to AIHA. We treated the patient by LDLT using an ABOi liver graft. The treatment strategy included anti-CD20 antibody, plasma exchange and transfusion before LDLT. The patient's anemia improved after surgery; she required only 2 units of irradiated red blood cell concentrates-leukocytes reduced. The patient was discharged from the hospital on postoperative day 35. Two years after surgery, the patient still shows normal hepatic and hematological findings. The immunomodulation protocol for ABOi LDLT was effective not only to avoid humoral reactions associated with ABOi LDLT, but also those associated with AIHA. PMID:21693332
Sanefuji, K; Ikegami, T; Nagata, S; Sugimachi, K; Gion, T; Uchiyama, H; Soejima, Y; Taketomi, A; Shirabe, K; Maehara, Y
Autologous blood transfusions (ABTs) has been used by athletes for approximately 4 decades to enhance their performance. Although the method was prohibited by the International Olympic Committee in the mid 1980s, no direct detection method has yet been developed and implemented by the World Anti-Doping Agency (WADA). Several indirect methods have been proposed with the majority relying on changes in erythropoiesis-sensitive blood markers. Compared with the first methods developed in 1987, the sensitivity of subsequent tests has not improved the detection of blood doping. Nevertheless, the use of sophisticated statistical algorithms has assured a higher level of specificity in subsequent detection models, which is a crucial aspect of antidoping testing particularly to avoid "false positives." Today, the testing markers with the best sensitivity/specificity ratio are the Hbmr model (an algorithm based on the total amount of circulating hemoglobin level [hemoglobin level mass] and percentage of reticulocytes, 4.51·ln(Hbmass)-?%ret) and the OFF-hr model (algorithm based on hemoglobin level concentration and percentage of reticulocytes, Hb(g/L)-60·?%ret). Only the OFF-hr model is currently approved by WADA. Recently, alternative indirect strategies for detecting blood doping have been proposed. One method is based upon a transfusion-induced immune-response resulting in specific changes in gene expression related to leukocytes such as T lymphocytes. Another method relies on detecting increased plasticizer metabolite levels in the urine caused by the leakage of plasticizers from the blood bags used during the blood storage. These methods need further development and validation across different types of transfusion regimes before they can be implemented. In addition, several research projects have been funded by WADA in recent years and are now under development including "Detection of Autologous Blood Transfusions Using Activated Red Blood Cells (the red blood cells eNOS system)" and "Detection of Autologous Blood Transfusion by Proteomic: Screening to find Unique Biomarkers, Detecting Blood Manipulation from Total Hemoglobin Mass using 15-nitric Oxide as a Tracer Gas, Storage Contamination as a Potential Diagnostic Test for Autologous Blood Transfusion and Test for Blood Transfusion (Autologous/Homologous) based on Changes of Erythrocyte Membrane Protome" (WADA, WADA Funded Research Projects. http://www.wada-ama.org/en/Science-Medicine/Research/Funded-Research-Projects/. 2010). Although strategies to detect autologous blood transfusion have improved, a highly sensitive test to detect small volumes of transfused autologous blood has not yet been implemented. PMID:22119492
Transfusion of banked donor erythrocytes can be life saving for small and ill neonates with severe anemia or active hemorrhage. However, risks of transfusions exist and must be weighed against potential benefits each time a transfusion is considered. The present review seeks to bring together the published data supporting 2 newly postulated risks of transfusions among very low-birth-weight neonates. The first is an association between "early" red blood cell transfusions, those administered in the first few days after birth, and the subsequent occurrence of a grade 3 or 4 intraventricular hemorrhage. The second is an association between "late" RBC transfusions and the subsequent occurrence of necrotizing enterocolitis. Much remains to be discovered about the pathogenetic links between transfusion and these adverse outcomes. Moreover, work is needed to clearly establish whether transfusions are causatively associated with these adverse outcomes or are covariables. The purpose of this chapter is to review the associations between transfusion and intraventricular hemorrhage and between transfusions and necrotizing enterocolitis and to use these associations to hypothesize that evidence-based improvements in transfusion practice have the potential to improve neonatal intensive care unit outcomes. PMID:22818549
Christensen, Robert D
This study was carried out to determine the effect of perioperative blood transfusions on the survival of patients operated on for colorectal cancers. Cox's regression analysis was applied to 281 patients operated for cure of Dukes' stage A, B or C disease. Other variables studied were age, sex, tumour location, and preoperative hemoglobin, lymphocyte and albumin values. Perioperative deaths, pre- and postoperative immunodepression, neoplasia in situ, nonresections and stage D disease were excluded. It was found that the number of units of blood transfused had a strong influence on the prognosis of patients with colorectal cancer, particularly colonic cancers, but the effect could not be demonstrated when rectal cancers were studied separately, perhaps because of the small number of cases. The mechanism of action of blood transfusions seems to be independent of the other analysed variables. The authors suggest that perioperative blood transfusions may have an immunomodulatory effect in patients with colonic cancer, as already shown in recipients of transfused kidney allografts. PMID:3756652
Corman, J; Arnoux, R; Péloquin, A; St-Louis, G; Smeesters, C; Giroux, L
The emergence of variant Creutzfeld-Jakob disease, following on from the bovine spongiform encephalopathy (BSE) epidemic, led to concerns about the potential risk of iatrogenic transmission of disease by blood transfusion and the introduction of costly control measures to protect blood supplies. We previously reported preliminary data demonstrating the transmission of BSE and natural scrapie by blood transfusion in sheep. The final results of this experiment, reported here, give unexpectedly high transmission rates by transfusion of 36% for BSE and 43% for scrapie. A proportion of BSE-infected transfusion recipients (3 of 8) survived for up to 7 years without showing clinical signs of disease. The majority of transmissions resulted from blood collected from donors at more than 50% of the estimated incubation period. The high transmission rates and relatively short and consistent incubation periods in clinically positive recipients suggest that infectivity titers in blood were substantial and/or that blood transfusion is an efficient method of transmission. This experiment has established the value of using sheep as a model for studying transmission of variant Creutzfeld-Jakob disease by blood products in humans. PMID:18647958
Houston, Fiona; McCutcheon, Sandra; Goldmann, Wilfred; Chong, Angela; Foster, James; Sisó, Silvia; González, Lorenzo; Jeffrey, Martin; Hunter, Nora
Background: Although red cell transfusions are lifesavers for patients with thalassemia, they are responsible for a series of complications and expose the patients to a variety of risks. Material and Methods: This cross-sectional study included 464 Egyptian beta(?) thalassemia major patients whose age ranged between 10 months and 31 years (mean 10.2 ± 6.6 years). All patients were subjected to thorough history taking with special emphasis on blood transfusions regarding rate of blood transfusion, type of received blood, and history of previous transfusion reactions in addition to type of chelation and compliance to iron chelation therapy and history of diabetes. Serum ferritin and pretransfusion hemoglobin assessment were done for all patients. Results: The mean pretransfusion hemoglobin level was 5.7 ± 1.16 g/dl. Allergic reactions were observed in 3.9% of the patients during the period of the study, while the history of previous allergic reaction was given by 72% of the patients. Deferiprone showed better compliance (58.6%) than deferoxamine (26.3%). The prevalence of diabetes was 10.1% among the studied group. On comparing diabetics to nondiabetics, serum ferritin, transfusion intervals, and age were statistically higher among diabetics (P<0.001). Conclusion: Lower pretransfusion hemoglobin and high rate of prevalence of diabetes, in addition to better compliance to deferiprone than deferoxamine, were detected among the patients.
Ragab, Lamis A.; Hamdy, Mona M.; Shaheen, Iman A.; Yassin, Rania N.
The lupus-prone New Zealand Black (NZB) strain uniquely develops a genetically imposed severe spontaneous autoimmune hemolytic anemia (AIHA) that is very similar to the corresponding human disease. Previous studies have mapped anti-erythrocyte Ab (AEA)-promoting NZB loci to several chromosomal locations, including chromosome 4; however, none of these have been analyzed with interval congenics. In this study, we used NZB.NZW-Lbw2 congenic (designated Lbw2 congenic) mice containing an introgressed fragment of New Zealand White (NZW) on chromosome 4 encompassing Lbw2, a locus previously linked to survival, glomerulonephritis, and splenomegaly, to investigate its role in AIHA. Lbw2 congenic mice exhibited marked reductions in AEAs and splenomegaly but not in anti-nuclear Abs. Furthermore, Lbw2 congenics had greater numbers of marginal zone B cells and reduced expansion of peritoneal cells, particularly the B-1a cell subset at early ages, but no reduction in B cell response to LPS. Analysis of a panel of subinterval congenic mice showed that the full effect of Lbw2 on AEA production was dependent on three subloci, with splenomegaly mapping to two of the subloci and expansions of peritoneal cell populations, including B-1a cells to one. These results directly demonstrated the presence of AEA-specific promoting genes on NZB chromosome 4, documented a marked influence of background genes on autoimmune phenotypes related to Lbw2, and further refined the locations of the underlying genetic variants. Delineation of the Lbw2 genes should yield new insights into both the pathogenesis of AIHA and the nature of epistatic interactions of lupus-modifying genetic variants. PMID:22371393
Scatizzi, John C; Haraldsson, Maria K; Pollard, K Michael; Theofilopoulos, Argyrios N; Kono, Dwight H
Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy with severe clinical manifestation, frequent recurrence, and poor long-term prognosis. It is usually caused by abnormalities in complement regulation. We report 2 cases of children affected by a catastrophic extrarenal complication. A 4-year-old Indian girl developed gangrene of the finger tips 2 days after initial presentation of aHUS. Factor H autoantibodies were identified. Renal function continued to decline despite daily plasma exchanges, and she was started on peritoneal dialysis 5 days after admission. The distal tips of the left hand remained gangrenous with a line of demarcation. Three weeks later, she did not return for follow-up and died at home because of dialysis-related complications. An Arabic girl developed end-stage renal disease due to aHUS in the fourth month after birth. A de novo activating C3 mutation was found. At age 9 months, she suddenly developed ischemic changes in fingers of both hands and several toes. The lesions progressed, and several finger tips became gangrenous despite intense plasma exchange therapy. The decision was made to administer complement blocking therapy with the C5 antibody eculizumab. All nonnecrotic digits rapidly regained perfusion. The 3 already gangrenous fingers healed with loss of the end phalanges. During maintenance, eculizumab aHUS activity subsided completely and some late recovery of renal function was observed. aHUS may present by thrombotic macroangiopathy of small peripheral arteries. Eculizumab appears effective in preserving tissue viability if administered before gangrene occurs and should be considered as first-line rescue therapy in such cases. PMID:23230076
Malina, Michal; Gulati, Ashima; Bagga, Arvind; Majid, Mohammad A; Simkova, Eva; Schaefer, Franz
The lupus-prone NZB strain uniquely develops a genetically imposed severe spontaneous autoimmune hemolytic anemia (AIHA) that is very similar to the corresponding human disease. Previous studies have mapped anti-erythrocyte Ab (AEA)-promoting NZB loci to several chromosomal locations, including chromosome 4, however, none of these have been analyzed with interval congenics. Here, we used NZB.NZW-Lbw2 congenic (designated Lbw2 congenic) mice containing an introgressed fragment of NZW on chromosome 4 encompassing Lbw2, a locus previously linked to survival, glomerulonephritis, and splenomegaly, to investigate the role in AIHA. Lbw2 congenic mice exhibited marked reductions in AEAs and splenomegaly, but not in anti-nuclear Abs. Furthermore, Lbw2 congenics had greater numbers of marginal zone B cells and reduced expansion of peritoneal cells, particularly the B-1a cell subset at early ages, but no reduction in B cell response to LPS. Analysis of a panel of subinterval congenic mice showed that the full effect of Lbw2 on AEA production was dependent on three subloci, with splenomegaly mapping to two of the subloci, and expansions of peritoneal cell populations, including B-1a cells to one. These results directly demonstrated the presence of AEA-specific promoting genes on NZB chromosome 4, documented a marked influence of background genes on autoimmune phenotypes related to Lbw2, and further refined the locations of the underlying genetic variants. Delineation of the Lbw2 genes should yield new insights into both the pathogenesis of AIHA and the nature of epistatic interactions of lupus-modifying genetic variants.
Scatizzi, John C.; Haraldsson, Maria K.; Pollard, K. Michael; Theofilopoulos, Argyrios N.; Kono, Dwight H.
The effects of environmental factors on a highly radiation-resistant hemolytic micrococcus isolated from chicken meat were studied. NaCl tolerance and gamma radiation resistance of the cells were growth phase-related. The cells were resistant to injury from drying or freezing/thawing. Under certain conditions, cells in the frozen state required approximately 5 Mrad to inactivate 90% of the population; 0.2 Mrad injured an equivalent proportion. Survival curve of the cells heated at 60/sup 0/C showed a unique pattern which was in three distinct phases. Heat-stressed cells were much more sensitive to radiation inactivation than unheated cells. When suspended in fresh m-Plate Count Broth (PCB), the injured cells repaired without multiplication during incubation at 32/sup 0/C. The repair process in this bacterium, however, was slower compared to thermally injured organisms studied by other workers. An improved replica-plating technique, was devised for isolation of radiation-sensitive mutants of pigmented bacteria. A simple method to demonstrate radiation-inducible radiation resistance in microbial cells was developed. The new method required neither washing/centrifugation nor procedures for cell enumeration. Mutagenesis treatment of radiation-resistant micrococcal bacterium with N-methyl-N'-nitro-N-nitrosoguanidine (NTG) followed by FPR and screening steps resulted in isolation of two radiation-sensitive mutants. The more sensitive mutant strain, designated as 702, was seven times as sensitive to gamma or UC radiation as the wild type. No apparent difference was observed between 702 and the wild type in (1) cell morphology, colonial morphology, and pigment production or (2) tolerance to NaCl, drying/storage, freezing/thawing, and heating. Sodium dodecyl sulfate treatment (for curing) of wild type did not result in isolation of a radiation-sensitive mutant.
Background The hemolytic activity of skin secretions obtained by stimulating the frog Kaloula pulchra hainana with diethyl ether was tested using human, cattle, rabbit, and chicken erythrocytes. The skin secretions had a significant concentration-dependent hemolytic effect on erythrocytes. The hemolytic activity of the skin secretions was studied in the presence of osmotic protectants (polyethylene glycols and carbohydrates), cations (Mg2+, Ca2+, Ba2+, Cu2+, and K+), or antioxidants (ascorbic acid, reduced glutathione, and cysteine). Results Depending on their molecular mass, osmotic protectants effectively inhibited hemolysis. The inhibition of skin hemolysis was observed after treatment with polyethylene glycols (1000, 3400, and 6000 Da). Among divalent cations, only 1 mM Cu2+ markedly inhibited hemolytic activity. Antioxidant compounds slightly reduced the hemolytic activity. Conclusions The results suggested that skin secretions of K. pulchra hainana induce a pore-forming mechanism to form pores with a diameter of 1.36-2.0 nm rather than causing oxidative damage to the erythrocyte membrane.
Polycyclic aromatic hydrocarbons (PAHs) in crude oil cause a range of adverse effects in oiled seabirds, one of the most common being hemolytic anemia via oxidative attack of erythrocytes by PAH metabolites resulting in hemoglobin leakage and formation of Heinz bodies. In such cases, haptoglobin and ferritin are up-regulated to sequester free Hb and iron in the circulation. We investigated these plasma proteins as biomarkers of PAH-induced Heinz body hemolytic anemia in oiled seabirds. Concentration ranges of PAHs, HAP and FT in plasma samples were 10-184 ng/ml, 0-2.6 mg/ml and 0-7.6 ng/ml, respectively. Dose-response relationships between plasma PAH exposure and haptoglobin and ferritin (FT) were investigated, and evidence of erythrocyte Heinz body formation studied in 50 oiled common guillemots stranded on the Norfolk Wash coast (East England). Haptoglobin was negatively correlated, and FT was positively correlated with PAH exposure. Heinz bodies were also observed confirming the toxic mechanism causing hemolytic anemia and counts were positively correlated with exposure. Our results support the application of these complementary biomarkers to assess hemolytic effects of oiling in wildlife biomonitoring, which also discriminate the influence of hemolytic versus inflammatory effects in oiled guillemots. PMID:17674967
Troisi, Gera; Borjesson, Lars; Bexton, Steve; Robinson, Ian
The religious organization of Jehovah's Witnesses numbers more than 7 million members worldwide, including 165,000 members in Germany. Although Jehovah's Witnesses strictly refuse the transfusion of allogeneic red blood cells, platelets and plasma, Jehovah's Witness patients may nevertheless benefit from modern therapeutic concepts including major surgical procedures without facing an excessive risk of death. The present review describes the perioperative management of surgical Jehovah's Witness patients aiming to prevent fatal anemia