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Sample records for histiocytosis orbital lesion

  1. Langerhans Cell Histiocytosis of the Orbit

    PubMed Central

    Herwig, Martina C.; Wojno, Ted; Zhang, Qing; Grossniklaus, Hans E.

    2014-01-01

    Langerhans cell histiocytosis (LCH) is a proliferation of Langerhans cells intermixed with inflammatory cells, in particular eosinophils, that may manifest as unisystem (unifocal or multifocal) or multisystem disease. Orbital involvement typically manifests as a solitary lesion that carries a favorable prognosis. Herein, we describe the clinical and histologic spectrum of LCH of the orbit on the basis of five cases. One patient exhibited multifocal unisystem disease, the other four patients presented with a localized process. The typical histologic features included numerous histiocytes with varying degrees of giant cell formation and scattered eosinophilic granulocytes. The presence of Langerhans cells was confirmed by CD1a and S100 immunostaining. Transmission electron microscopy exhibited characteristic intracytoplasmic Birbeck granules. The different ophthalmic manifestations of LCH and treatment strategies are reviewed in the context of previously reported cases. As LCH may solely involve the orbit, treatment is based on the degree of organ involvement. LCH has to be included in the differential diagnosis in tumors of the ocular adnexae, in particular in young children. PMID:23246282

  2. Histiocytosis

    MedlinePlus

    Histiocytosis X; Langerhans cell histiocytosis; Eosinophilic granuloma; Pulmonary histiocytosis X; Nonlipid reticuloendotheliosis; Pulmonary Langerhans cell granulomatosis; Hand-Schuller-Christian ...

  3. Histiocytosis

    MedlinePlus

    Histiocytosis X; Langerhans cell histiocytosis; Eosinophilic granuloma; Pulmonary histiocytosis X; Nonlipid reticuloendotheliosis; Pulmonary Langerhans cell granulomatosis; Hand-Schuller-Christian disease; Letterer-Siwe disease

  4. Langerhans cell histiocytosis with presentation as orbital disease

    PubMed Central

    Bhanage, Ashok B.; Katkar, Anand D.; Ghate, Prajakta S.

    2015-01-01

    Langerhans cell histiocytosis (LCH) is an uncommon multisystem disease with an abnormal polyclonal proliferation of Langerhans cells that invade various organs. In rare instances, the affection of the orbit is the only and the first symptom. We report an unusual case of an 18-month-old male who presented with orbital disease as the first symptom, in the form of chronic presentation of periorbital swelling (2 months duration) with acute inflammation (1-week duration) giving a suspicion of orbital cellulitis. Histopathology after radical excision confirmed the diagnosis of LCH and was advised initial therapy as per Histiocyte Society Evaluation and Treatment Guidelines (2009) but was lost to follow-up only reappearing with progression (multisystem LCH with risk organ involvement) and developed progressive active disease on treatment after 5 weeks. He was treated with salvage therapy for risk patients achieving complete remission. PMID:26167225

  5. Histiocytosis X revealed by diabetes insipidus and skin lesions.

    PubMed

    El Fekih, Nadia; Kamoun, Inés; Jones, Meriem; Remmeh, Soummeya; Zéglaoui, Faten; Ben Slama, Claude; Fazaa, Bécima

    2013-07-01

    Langerhans cell histiocytosis is part of a larger group of syndromes described as histiocytoses. The disease may involve single or multiple systems including skin and nervous system. Here we report an adult case where Langerhans cell histiocytosis presented with diabetes insipidus and cutaneous ulcers. PMID:23538895

  6. Crystal storing histiocytosis presenting as a temporal lobe mass lesion

    PubMed Central

    Johnson, Mahlon; Mazariegos, Juan; Lewis, P. Jeffery; Pomakova, Diana

    2013-01-01

    Background: Crystal storing histiocytosis (CSH) is a disorder characterized by local or diffuse infiltration of histiocytes containing crystalline inclusions most commonly of immunoglobulin light chain. Involvement of the central nervous system is extremely rare. CSH may be misdiagnosed as an infection or tumor. In patients with involvement of other organs, it is frequently associated with lymphoplasmacytic diseases. Case Description: A 20-year-old female was evaluated for 2 weeks of progressively worsening headaches. At presentation, she had no history of fevers but reported a sore throat without cough 3-4 days prior. Her past medical history was unremarkable. She denied intravenous drug use or sexually transmitted diseases but lived with an individual with a history of fungal meningitis. On examination she was afebrile, alert, and oriented with a blood pressure of 110/70 mmHg. She had no adenopathy or neurological deficits. Her white blood cell count was minimally elevated. Magnetic resonance imaging revealed a 3.5 × 1.3 × 1.9 cm contrast enhancing lesion of the left temporal lobe with a mild midline shift. Evaluation by multiple specialists suggested a differential diagnosis of an infectious or neoplastic process. Cultures for infectious agents were negative. The biopsy showed CSH. Postoperatively and at 1 month follow up, she was neurologically intact. Conclusion: Radiographically and intraoperatively, CSH may mimic an infectious process or neoplasm. Its recognition is critical to facilitate appropriate therapy and prompt screening for an occult lymphoplasmacytic neoplasm, plasma cell dyscrasia or other underlying disease. PMID:24032087

  7. Dendritic Cells Cause Bone Lesions in a New Mouse Model of Histiocytosis

    PubMed Central

    Grosjean, Frédéric; Nasi, Sonia; Schneider, Pascal; Chobaz, Véronique; Liu, Alexandra; Mordasini, Vanessa; Moullec, Kristell; Vezzoni, Paolo; Lavanchy, Christine; Busso, Nathalie; Acha-Orbea, Hans; Ehirchiou, Driss

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare disease caused by the clonal accumulation of dendritic Langerhans cells, which is often accompanied by osteolytic lesions. It has been reported that osteoclast-like cells play a major role in the pathogenic bone destruction seen in patients with LCH and these cells are postulated to originate from the fusion of DCs. However, due to the lack of reliable animal models the pathogenesis of LCH is still poorly understood. In this study, we have established a mouse model of histiocytosis- recapitulating human disease for osteolytic lesions seen in LCH patients. At 12 weeks after birth, severe bone lesions were observed in our multisystem histiocytosis (Mushi) model, when CD8α conventional dendritic cells (DCs) are transformed (MuTuDC) and accumulate. Most importantly, our study demonstrates that bone loss in LCH can be accounted for the transdifferentiation of MuTuDCs into functional osteoclasts both in vivo and in vitro. Moreover, we have shown that injected MuTuDCs reverse the osteopetrotic phenotype of oc/oc mice in vivo. In conclusion, our results support a crucial role of DCs in bone lesions in histiocytosis patients. Furthermore, our new model of LCH based on adoptive transfer of MuTuDC lines, leading to bone lesions within 1–2 weeks, will be an important tool for investigating the pathophysiology of this disease and ultimately for evaluating the potential of anti-resorptive drugs for the treatment of bone lesions. PMID:26247358

  8. Radiotherapy in benign orbital disease. II: Ophthalmic Graves' disease and orbital histiocytosis X.

    PubMed Central

    Harnett, A. N.; Doughty, D.; Hirst, A.; Plowman, P. N.

    1988-01-01

    Ophthalmic Graves' disease and histiocytosis X involving the orbit are occasionally refractory to treatment, so that vision may be threatened. In these situations megavoltage external beam radiotherapy should be employed, and the indications for this treatment are discussed. A highly accurate technique is described, using precise planning with information obtained from high definition CT scans, a complete patient head shell for immobilisation, and modern megavoltage radiotherapy treatment machines. As a result the dose to the lens is minimised (to a maximum of 10% of the prescribed dose), and late morbidity will be small. Two cases are described to illustrate this procedure and the response to treatment. Images PMID:3259894

  9. Relative efficacy of radiographic and radionuclide bone surveys in the detection of the skeletal lesions of histiocytosis X

    SciTech Connect

    Parker, B.R.; Pinckney, L.; Etcubanas, E.

    1980-02-01

    Radionuclide studies have been considered a more sensitive indicator of the presence of the bony lesions of histiocytosis X in children that have radiographic bone surveys. Our results suggest that the opposite is true. Although positive correlation between bone scans and radiographs was found in eight of nine patients, only 35% of the individual lesions visible on radiographs were seen on radionuclide studies. Results of radionuclide studies did not correlate with age or sex of the patient, presence or duration of symptoms, radiographic appearance, or anatomic location of lesions. Our results suggest that the radiographic skeletal survey is more sensitive than radionuclide studies in detecting the skeletal lesions of histiocytosis X.

  10. Irradiation of the hypothalamic-hypophyseal area induces complete regression of mucocutaneous lesions in disseminated histiocytosis X

    SciTech Connect

    Palmieri, G.; Stefani, S.; Gridelli, C.; Conte, A.; Airoma, G.; Contegiacomo, A.; Bianco, A.R.

    1989-07-01

    We report on a 54-year-old woman with disseminated histiocytosis X who had a complete regression of all mucocutaneous lesions within 1 month from the completion of radiation therapy (4500 cGy) to the hypothalamic-hypophyseal (H-H) area. This response lasted 12 months, after which new cutaneous and bone lesions appeared.

  11. Polyclonal T-Cells Express CD1a in Langerhans Cell Histiocytosis (LCH) Lesions

    PubMed Central

    West, Jennifer A.; Olsen, Sharon L.; Mitchell, Jenée M.; Priddle, Ross E.; Luke, Jennifer M.; Åkefeldt, Selma Olsson; Henter, Jan-Inge; Turville, Christopher; Kannourakis, George

    2014-01-01

    Langerhans cell histiocytosis (LCH) is a complex and poorly understood disorder that has characteristics of both inflammatory and neoplastic disease. By using eight-colour flow cytometry, we have identified a previously unreported population of CD1a+/CD3+ T-cells in LCH lesions. The expression of CD1a is regarded as a hallmark of this disease; however, it has always been presumed that it was only expressed by pathogenic Langerhans cells (LCs). We have now detected CD1a expression by a range of T-cell subsets within all of the LCH lesions that were examined, establishing that CD1a expression in these lesions is no longer restricted to pathogenic LCs. The presence of CD1a+ T-cells in all of the LCH lesions that we have studied to date warrants further investigation into their biological function to determine whether these cells are important in the pathogenesis of LCH. PMID:25343480

  12. Relapsing nodular lesions in the course of adult pulmonary Langerhans cell histiocytosis.

    PubMed

    Tazi, A; Montcelly, L; Bergeron, A; Valeyre, D; Battesti, J P; Hance, A J

    1998-06-01

    In most patients with pulmonary Langerhans cell histiocytosis (LCH), clinical and radiological abnormalities initially either stabilize or regress, often without treatment. Little information is available, however, concerning the subsequent evolution of disease in patients who initially follow a benign course. We describe four patients with biopsy-confirmed pulmonary LCH whose initial course was characterized by regression of parenchymal nodular lesions, but who subsequently developed one or more episodes of active disease 7 mo to 7.5 yr after their initial presentation. In each case, the subsequent episodes of active disease were characterized by the reappearance or marked increase in nodular radiographic abnormalities, whose presence was confirmed by high-resolution computed tomography (HRCT). Thus, initial regression of nodular lesions in pulmonary LCH does not preclude the reappearance of one or more episodes of active disease, and may have important consequences on the long-term prognosis of these patients. PMID:9620939

  13. Histiocytosis X.

    PubMed

    Favara, B E; McCarthy, R C; Mierau, G W

    1983-08-01

    To clarify salient issues pertaining to histiocytosis X--a syndrome that includes Letterer-Siwe disease, Hand-Schuller-Christian disease, and eosinophilic granuloma--the authors review the epidemiologic data and the histologic, morphologic, and clinical bases for diagnosis and prognosis. Histiocytes are defined and their possible histogenesis outlined, and Langerhans cells, which may be a leading element in active lesions, are characterized. The authors outline hypothetic pathogenetic schema, which they recommend be tested by recently developed immunologic and genetic means, since histiocytosis X, at least in its disseminated form, remains an unpredictable disease for which there is no proven effective therapy. PMID:6347865

  14. Endoscopic management of orbital apex lesions.

    PubMed

    Sethi, D S; Lau, D P

    1997-01-01

    Lesions of the orbital apex often present a diagnostic dilemma. Clinical assessment and imaging studies are helpful but a tissue biopsy is often required. The morbidity associated with transcranial approaches to the orbital apex may outweigh the benefits of obtaining a biopsy by these routes. Fine needle aspiration cytology of orbital apex lesions can be performed but there are disadvantages with this method. We describe a transnasal endoscopic technique to biopsy the orbital apex. The technique was used successfully to obtain a tissue diagnosis in six patients with orbital apex lesions. This enabled commencement of definitive treatment. There were no significant complications. The transnasal approach to the orbital apex using the endoscopes is reliable. Endoscopes provide excellent illumination, magnification, and a panoramic view of the operative field. PMID:9438058

  15. Neither IL-17A mRNA Nor IL-17A Protein Are Detectable in Langerhans Cell Histiocytosis Lesions

    PubMed Central

    Peters, Tricia L; McClain, Kenneth L; Allen, Carl E

    2011-01-01

    Langerhans cell histiocytosis (LCH) is a rare disease characterized by heterogeneous lesions including CD207+/CD1a+ dendritic cells that can result in significant morbidity and mortality. The etiology of LCH remains speculative, and neoplastic and inflammatory origins have been debated for decades. A recent study identified abundant interleukin-17 (IL-17A) protein in dendritic cells in LCH lesions as well as in plasma from patients with active disease. Furthermore, it identified dendritic cells as a novel source of IL-17A expression. However, subsequent studies from our research group failed to identify any IL-17A gene expression from CD207+ dendritic cells or CD3+ T cells in LCH lesions. In this study, further investigation once again fails to identify any cells in LCH lesions with IL-17A gene expression. Furthermore, IL-17A antigen is undetectable in LCH lesion lysates with western blotting, immunoprecipitation, spectral analysis, and enzyme-linked immunosorbent assay (ELISA). Western blots, immunoprecipitation, and ELISA experiments also demonstrate that antibodies used in original studies that established the IL-17A hypothesis for pathogenesis of LCH recognize nonspecific proteins. We conclude that evidence for IL-17A as a significant factor in LCH remains inadequate and clinical trials targeting IL-17A remain unjustified. PMID:21654633

  16. Insufficiency of Bone Scintigraphy in Vertebral Lesions of Langerhans Cell Histiocytosis Compared to F-18 Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography and Diagnostic Computed Tomography

    PubMed Central

    Koç, Zehra Pınar; Şimşek, Selçuk; Akarsu, Saadet; Balcı, Tansel Ansal; Onur, Mehmet Ruhi; Kepenek, Ferat

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a benign disorder related to the histiocytes which can infiltrate bone tissue. The most effective method for demonstrating severity of this disease is PET/CT and bone scintigraphy might show bone lesions. We present a seventeen year old male patient with disseminated LCH presented with exophtalmos and having multiple vertebral lesions which were identified by F-18 FDG PET/CT scan and diagnostic CT but not in the bone scintigraphy. PMID:25800594

  17. Sclerosing Lesions of the Orbit: A Review

    PubMed Central

    Lokdarshi, Gautam; Pushker, Neelam; Bajaj, Mandeep S.

    2015-01-01

    Orbital sclerosing inflammation is a distinct group of pathologies characterized by indolent growth with minimal or no signs of inflammation. However, contrary to earlier classifications, it should not be considered a chronic stage of acute inflammation. Although rare, orbital IgG4-related disease has been associated with systemic sclerosing pseudotumor-like lesions. Possible mechanisms include autoimmune and IgG4 related defective clonal proliferation. Currently, there is no specific treatment protocol for IgG4-related disease although the response to low dose steroid provides a good response as compared to non-IgG4 sclerosing pseudotumor. Specific sclerosing inflammations (e.g. Wegener's disease, sarcoidosis, Sjogren's syndrome) and neoplasms (lymphoma, metastatic breast carcinoma) should be ruled out before considering idiopathic sclerosing inflammation as a diagnosis. PMID:26692715

  18. Activated Conventional T-Cells Are Present in Langerhans Cell Histiocytosis Lesions Despite the Presence of Immune Suppressive Cytokines.

    PubMed

    Quispel, Willemijn T; Stegehuis-Kamp, Janine A; Santos, Susy J; Egeler, R Maarten; van Halteren, Astrid G S

    2015-10-01

    Langerhans cell histiocytosis (LCH) lesions are characterized by neoplastic CD1a(+)/Langerin(+) histiocytes (LCH-cells) and display many features of chronic inflammation. Cancer cells can escape immune-surveillance through intra-tumoral secretion of immune-suppressive cytokines. We therefore studied by immunohistochemistry the local cytokine milieu and phenotypic characteristics of T-cells and LCH-cells present in LCH lesions collected from 25 therapy naïve patients. LCH biopsies predominantly expressed interleukin-10 (IL-10) (10/25), transforming growth factor-beta (TGF-β) (9/25), or both cytokines (6/25). The absolute number of CD3(+)T-cells and the CD3(+)FOXP3(-) conventional cell (T-CONV) versus the CD3(+)FOXP3(+) regulatory T-cell (T-REG) was comparable for each suppressive cytokine profile (5:1). IL-10-expressing lesions contained, however, a higher proportion of T-CONV expressing the activation markers CD25 98% (38%-100%) and inducible costimulatory molecule (ICOS) 86% (47%-100%) than lesions wherein solely TGF-β was detected (CD25(+) 20% (6%-54%); ICOS(+) 29% (7%-51%)). Virtually all T-REG expressed CD25 and ICOS in IL-10 lesions, whereas TGF-β(+) lesions contained a lower proportion of ICOS(+) T-REG (P=0.05). IL-10(+) lesions contained more LCH-cells expressing high intensity of ICOS ligand (ICOSL) compared with TGF-β(+) lesions (P=0.03). ICOS expression by lesion-infiltrating T-CONV and T-REG positively correlated to the extent of ICOSL expression by LCH-cells (P=0.004). Our study points out that the combined detection of interlesional IL-10 and ICOSL expression by LCH-cells is associated with the highest prevalence of activated T-CONV. Immune profiling of LCH-affected tissues obtained at the time of diagnosis may set the stage for the development of new types of therapies, which aim at local boosting of immune cells that recognize and eliminate neoplastic LCH-cells. PMID:26381039

  19. Reactive histiocytosis of the orbit and posterior segment in a dog.

    PubMed

    Pumphrey, Stephanie A; Pizzirani, Stefano; Pirie, Christopher G; Sato, Amy F; Buckley, Faith I

    2013-05-01

    We present a case of reactive histiocytic disease involving the orbit, optic nerve, retina, and choroid in a Border Collie dog initially presenting for vision loss. Long-term partial return of vision has been achieved with systemic immunosuppression. Anterior segment and ocular surface manifestations of reactive histiocytic disease in dogs are relatively common. Posterior segment and orbital involvement, however, are minimally documented in the existing literature. To the authors' knowledge, this is the first report of disease confined to the orbit and posterior segment as well as the first report of vision loss as a presenting complaint for reactive histiocytic disease. Clinical, magnetic resonance imaging, cytologic, and histopathologic findings are reviewed. PMID:22853429

  20. Orbital lesions: proton spectroscopic phase-dependent contrast MR imaging.

    PubMed

    Atlas, S W; Grossman, R I; Axel, L; Hackney, D B; Bilaniuk, L T; Goldberg, H I; Zimmerman, R A

    1987-08-01

    Thirteen orbital lesions in 12 patients were evaluated with both conventional spin-echo magnetic resonance (MR) imaging and phase-dependent proton spectroscopic imaging. This technique, which makes use of small differences in the resonant frequencies of water and fat protons, provides excellent high-resolution images with simultaneous chemical shift information. In this method, there is 180 degrees opposition of phase between fat protons and water protons at the time of the gradient echo, resulting in signal cancellation in voxels containing equal signals from fat and water. In this preliminary series, advantages of spectroscopic images in orbital lesions included better lesion delineation, with superior anatomic definition of orbital apex involvement; more specific characterization of high-intensity hemorrhage with a single pulse sequence; elimination of potential confusion from chemical shift misregistration artifact; further clarification of possible intravascular flow abnormalities; and improved apparent intralesional contrast. PMID:3602394

  1. Histiocytosis X in the lung.

    PubMed

    Colby, T V; Lombard, C

    1983-10-01

    The histologic and clinical features of pulmonary histiocytosis X are discussed. The majority of patients are in the third and fourth decades of life. They present with nonspecific respiratory complaints and bilateral reticulonodular infiltrates that can be seen on chest radiographs. The histologic lesions of pulmonary histiocytosis X are scattered, discrete nodules that frequently center on small airways. The initially cellular nodules are replaced progressively by fibrous tissue. The cell infiltrates in active lesions contain variable numbers of eosinophils and histiocytes resembling Langerhans cells of the epidermis (Hx cells). In most patients the disease resolves or stabilizes, leaving few, if any, significant residues. A minority of patients develop progressive pulmonary disease that is ultimately fatal. The cause of pulmonary histiocytosis X is unknown, but two recent series suggest a relationship to cigarette smoking. PMID:6352456

  2. A case of Langerhans cell histiocytosis of the skull in which preoperative methionine positron emission tomography was useful in comprehending the spreading of the lesion

    PubMed Central

    Yamaki, Tetsu; Kokubo, Yasuaki; Saito, Yuki; Matsuda, Kenichiro; Funiu, Hayato; Sakurada, Kaori; Sato, Shinya; Kayama, Takamasa

    2014-01-01

    Background: The problem with treatments against skull lesions of Langerhans cell histiocytosis (LCH) is that invasions often reach the bones and dura mater, making it difficult to accurately comprehend the range thereof prior to surgery. We herein report that 11C-methionine positron emission tomography (PET) (Met-PET) carried out prior to surgery was useful in comprehending the spreading of the lesion. Case Description: A 20-year-old female presented with swelling and dull pain on the left side of the head. A slightly heterogeneously reinforced tumor was observed inside the bone defect in the gadolinium-enhanced T1-wighted image upon magnetic resonance imaging (MRI) and the dura mater contacting the tumor was observed with an enhancing effect. Accumulation was poor in the center of the tumor upon Met-PET, and accumulation with a well-defined border was observed in the border thereof as well as the area adjacent to the brain. Surgical resection was performed; the pathological diagnosis was LCH. An invasion of tumor cells was observed in the dura mater with accumulation observed according to Met-PET. Moreover, the accumulation of tumor cells was observed in the area observed with accumulation inside the bone; however, the center part with poor accumulation lacked tumor cells, with fibrous tissue accounting for most parts. Conclusion: Met-PET was believed to be helpful in comprehending the spreading of the tumor in the surroundings of the brain surface for skull lesions. PMID:24778915

  3. Sinus histiocytosis with massive lymphadenopathy

    SciTech Connect

    Pastakia, B.; Weiss, S.H.

    1987-11-01

    Gallium uptake corresponding to the extent of the disease in a patient with histologically proven sinus histiocytosis with massive lymphadenopathy (SHML) is reported. Computerized tomography confirmed the presence of bilateral retrobulbar masses, involvement of both lateral recti, erosion of the bony orbital floor with encroachment of tumor into the right maxillary antrum, and retropharyngeal involvement.

  4. Pericardial Effusion in Langerhans Cell Histiocytosis: A Case Report

    PubMed Central

    Gholami, Narges

    2016-01-01

    Introduction Langerhans cell histiocytosis (LCH) is a proliferative disorder of histiocytes in multiple organs. Langerhans cell histiocytosis involves bones, skin, lung and other organs. Case Presentation This study describes a seven-month-old Iranian girl who presented with skin rash and cervical lymphadenopathy. Langerhans cell histiocytosis was suspected when it was associated with anemia, splenomegaly and lytic bone lesions. A skin biopsy confirmed the diagnosis of Langerhans cell histiocytosis. During hospitalization, the patient looked ill with respiratory distress. A chest X-ray showed a ground glass view, and echocardiography showed moderate pericardial effusion. Conclusions Pericardial effusion was a rare finding in this case of Langerhans cell histiocytosis. Pericardial effusion in Langerhans cell histiocytosis, which is an unusual presentation, should be considered when the patient experiences respiratory distress. PMID:27621925

  5. Langerhans' cell histiocytosis of the temporal fossa: A case report

    PubMed Central

    LIANG, CHEN; LIANG, QIANLEI; DU, CHANGWANG; ZHANG, XIAODONG; GUO, SHIWEN

    2016-01-01

    Langerhans' cell histiocytosis (LCH) is a rare disease with a wide spectrum of clinical manifestations, varying from an isolated lesion to systemic involvement. The etiology of this disease remains to be elucidated. The present study reports a case of LCH with temporal fossa localization in an 8-year-old male patient, who had exhibited left temporal pain and headache for 1 month. Physical examination revealed slight exophthalmos and conjunctival hemorrhage in the patient's left eye, and non-contrast computed tomography imaging of the head revealed a soft tissue mass with unclear margins located in the left temporal fossa, as well as a wide bony defect. Magnetic resonance imaging revealed a heterogeneously contrast-enhanced mass near the left temporal pole, which eroded into the patient's left orbit and maxillary sinus. The lesion was totally excised and confirmed to be LCH through biopsy. PMID:27073529

  6. Langerhans' cell histiocytosis in an adult.

    PubMed

    Lindelöf, B; Forslind, B; Hilliges, M; Johansson, O; Aström, L

    1991-01-01

    A woman with typical skin lesions of histiocytosis X is reported. Electron microscopic and immunohistochemical investigations revealed a large number of markedly long Birbeck Langerhans' cell granulae. During treatment with Interferon alpha -2b, the patient developed infarctus cerebri and died. PMID:1675535

  7. [Histiocytosis X].

    PubMed

    Savich, S F; Pustovoĭ, V V; Rubinshteĭn, S Ia

    1982-01-01

    Letterer-Siwe disease in a girl of 1 1/2 months is described. The disease ran a course with predominant involvement of the organs of immunogenesis and hyperbilirubinemia. At the end of the disease most prominent signs consisted in suppurative-necrotic lesions of the skin and pneumonia. PMID:7082203

  8. Langerhans cell histiocytosis: a 10-year review.

    PubMed

    Shian, W J; Shu, S G; Chu, H Y; Chi, C S

    1994-01-01

    Hospital records of seventeen patients (11 males & 6 females) with Langerhans cell histiocytosis, confirmed by electron microscopic demonstration of Birbeck granules, were studied retrospectively from October 1982 to October 1992 at Taichung Veterans General Hospital. The ages at presentation ranged from 5 months to 17 years (a median of 6 years). The clinical features were protean and included fever, pain, bony lesions, lung lesions, abnormal dentition, diabetes insipidus, oral ulcer, otorrhea, dermatitis, anemia, thrombocytopenia, lymphadenopathy, and hepatosplenomegaly. Skull and femur were the most common sites of bony lesions. The main therapeutic modalities were excision, radiotherapy, and chemotherapy with vincristine and prednisolone. The young age at presentation, several involved organ systems, presence of organ dysfunction, and clinical diagnosis with Letterer-Siwe disease were poor prognostic factors. Although Langerhans cell histiocytosis is not a rare disease, the cause is still unknown. It needs further research to disclose the mystery. PMID:7942024

  9. Orbital masses: CT and MRI of common vascular lesions, benign tumors, and malignancies

    PubMed Central

    Khan, Sarah N.; Sepahdari, Ali R.

    2012-01-01

    A wide variety of space occupying lesions may be encountered in the orbit. CT and MR imaging frequently help confirm the presence of a mass and define its extent. Characteristic imaging features may help distinguish among lesions that have overlapping clinical presentations. This review focuses on some of the common orbital masses. Common vascular lesions that are reviewed include: capillary (infantile) hemangioma, cavernous hemangioma (solitary encapsulated venous-lymphatic malformation), and lymphangioma (venous-lymphatic malformation). Benign tumors that are reviewed include: optic nerve sheath meningioma, schwannoma, and neurofibroma. Malignancies that are reviewed include: lymphoma, metastasis, rhabdomyosarcoma, and optic glioma. Key imaging features that guide radiological diagnosis are discussed and illustrated. PMID:23961022

  10. Pulmonary langerhans cell histiocytosis

    PubMed Central

    2012-01-01

    Pulmonary Langerhans Cell Histiocytosis (PLCH) is a relatively uncommon lung disease that generally, but not invariably, occurs in cigarette smokers. The pathologic hallmark of PLCH is the accumulation of Langerhans and other inflammatory cells in small airways, resulting in the formation of nodular inflammatory lesions. While the overwhelming majority of patients are smokers, mechanisms by which smoking induces this disease are not known, but likely involve a combination of events resulting in enhanced recruitment and activation of Langerhans cells in small airways. Bronchiolar inflammation may be accompanied by variable lung interstitial and vascular involvement. While cellular inflammation is prominent in early disease, more advanced stages are characterized by cystic lung destruction, cicatricial scarring of airways, and pulmonary vascular remodeling. Pulmonary function is frequently abnormal at presentation. Imaging of the chest with high resolution chest CT scanning may show characteristic nodular and cystic abnormalities. Lung biopsy is necessary for a definitive diagnosis, although may not be required in instances were imaging findings are highly characteristic. There is no general consensus regarding the role of immunosuppressive therapy in smokers with PLCH. All smokers must be counseled on the importance of smoking cessation, which may result in regression of disease and obviate the need for systemic immunosuppressive therapy. The prognosis for most patients is relatively good, particularly if longitudinal lung function testing shows stability. Complications like pneumothoraces and secondary pulmonary hypertension may shorten life expectancy. Patients with progressive disease may require lung transplantation. PMID:22429393

  11. [Pneumothorax secondary to pulmonary histiocytosis X].

    PubMed

    Bianchi, M; Cataldi, M

    1999-01-01

    The authors report a case of pneumothorax in a young man suffering from pulmonary histiocytosis. They take this observation as a starting point for discussing about the so-called "histiocytosis X". The term "histiocytosis" is a term for a variety of proliferative disorders of histiocytes or macrophages. There is a small cluster of conditions characterized by proliferation of a special type of histiocyte called the "Langerhans' cell". The proliferation of Langerhans' cells results from disturbances in immunoregulation. Particularly, there are three clinicopathologic entities: *Letterer-Siwe disease (acute disseminated Langerhans' cells histiocytosis), *multifocal eosinophilic granuloma or Hand-Schüller-Christian disease (multifocal Langerhans' cells histiocytosis), *unifocal eosinophilic granuloma (unifocal Langerhans' cells histiocytosis). In the past, these were collected in the not specific term of histiocytosis X. Most patients with Langerhans' cell granulomatosis have diseases limited to the lung, only a small proportion have extrapulmonary involvement or a systemic disorder. Typically, there are expanding, erosive accumulations of Langerhans' cells within the medullary cavities of bones. In this reported case, a patient, two years before, was affected by osteolytic bone lesion in the left femur. Pulmonary histiocytosis is often seen in smokers in the third and fourth decade of life. The 20% of patients are asymptomatic and found to have an abnormal chest radiograph; a lesser proportion presents with pneumothorax. Some patients develop progressive fibrotic lung disease and respiratory failure. Chest radiographs may show bilateral reticular or reticulonodular infiltrates, sometimes with cystic changes, often with sparing of the costophrenic angles, but high resolution computerized tomography scans may be virtually diagnostic based on the predilection of the cysts and nodules for the upper lung zones. Sometimes the typical Langerhans' cells may be recovered in

  12. Prognostic factors in histiocytosis X.

    PubMed

    Lahey, M E

    1981-01-01

    It is now clear that the prognosis in children with histiocytosis X has improved considerable over the past few years. To be sure, patients with solitary lesions have an excellent prognosis. Whereas the outlook for patients with significant visceral involvement is not as good as those with bone lesions only, the outlook is by no means hopeless, as was once thought. A number of prognostic factors have been reviewed here. The most significant of these factors at the present time would appear to be age of onset of the disease, extent of involvement, the rapidity of progression of the disease, and, in particular, the presence or absence of dysfunction of such crucial organ systems as liver, lung, and hemopoietic system. Further studies of the significance of histologic features and immunologic findings are clearly needed to further our understanding of this disorder. PMID:6972178

  13. Langerhans Cell Histiocytosis: Emerging Insights and Clinical Implications.

    PubMed

    Zinn, Daniel J; Chakraborty, Rikhia; Allen, Carl E

    2016-02-01

    Langerhans cell histiocytosis is a disorder characterized by lesions that include CD207+ dendritic cells along with an inflammatory infiltrate. Langerhans cell histiocytosis has a highly variable clinical presentation, ranging from a single lesion to potentially fatal disseminated disease. The uncertainty as to whether Langerhans cell histiocytosis is a reactive or a neoplastic disease has resulted in a long-standing debate on this question, and the limited understanding of the pathogenesis of the disease has impeded clinical improvement for patients. The current standard of care for multisystem Langerhans cell histiocytosis, empirically derived chemotherapy with vinblastine and prednisone, cures fewer than 50% of patients, and optimal therapies for relapse and neurodegenerative disease remain uncertain. Recent research advances support a model in which Langerhans cell histiocytosis arises due to pathologic activation of the mitogen-activated protein kinase (MAPK) pathway in myeloid precursors. Redefinition of Langerhans cell histiocytosis as a myeloid neoplastic disorder driven by hyperactive ERK supports the potential of chemotherapy with efficacy against immature myeloid cells, as well as mutation-specific targeted therapy. PMID:26888790

  14. [Pulmonary manifestations of Langerhans cell histiocytosis].

    PubMed

    Obert, J; Tazi, A

    2015-10-01

    Pulmonary Langerhans cell histiocytosis is a rare diffuse cystic interstitial pneumonia of unknown etiology that occurs selectively in young smokers of both genders. The multicenter studies conducted by the reference center have better defined the short and medium terms natural history of the disease and the clinical management of patients. A substantial proportion of patients experience a dramatic decline in their lung function soon after diagnosis. Importantly, smoking cessation is associated with a decreased risk of subsequent deterioration. Cladribine, a purine analogue, chemotherapy may dramatically improve lung function in patients with progressive pulmonary Langerhans cell histiocytosis, but this treatment should be used only in the setting of clinical research. Specific pulmonary hypertension therapies (anti-endothelin receptors, inhibitors of phosphodiesterases) may be used with caution in specialized centres for patients with severe pulmonary hypertension, and seem to be well tolerated. The recent identification of the V600E mutation of the BRAF oncogene in approximately half of the Langerhans cell histiocytosis lesions, including pulmonary granulomas, represents an important step forward in the understanding of the pathogenesis of Langerhans cell histiocytosis. Potentially it opens the way to targeted therapies. PMID:26003197

  15. [Case for diagnosis. Langerhans cell histiocytosis].

    PubMed

    Mosqueira, Carolina Balbi; Xavier, Augusto Frederico de Paula; Tuschinski, Cíntia Letícia; Pinto, Clóvis A Lopes; Cunha, Paulo R

    2010-01-01

    Langerhans cell histiocytosis is a rare disease of unknown cause, characterized by the proliferation of histiocytic cells (Langerhans cells). Its diagnosis is especially difficult due to its wide clinical spectrum, ranging from a single lesion to a multisystemic disorder. Diagnosis may be confirmed by means of an immunohistochemical study. Treatment depends upon the severity of the disease, and systemic chemotherapy may be needed. PMID:20464099

  16. A Rare Case of Erdheim-Chester Disease and Langerhans Cell Histiocytosis Overlap Syndrome

    PubMed Central

    Nabi, Shahzaib; Arshad, Adeel; Jain, Tarun; Virk, Fawad; Gulati, Rohit; Awdish, Rana

    2015-01-01

    A 48-year-old woman with a past medical history of seizures and end-stage renal disease secondary to obstructive uropathy from retroperitoneal fibrosis presented to the emergency department with seizures and altered mental status. A Glasgow Coma Scale of 4 prompted intubation, and she was subsequently admitted to the intensive care unit. Magnetic resonance imaging of the brain performed to elucidate the aetiology of her seizure showed a dural-based mass within the left temporoparietal lobe as well as mass lesions within the orbits. Further imaging showed extensive retroperitoneal fibrosis extending to the mediastinum with involvement of aorta and posterior pleural space. Imaging of the long bones showed bilateral sclerosis and cortical thickening of the diaphyses. Imaging of the maxillofacial structures showed osseous destructive lesions involving the mandible. These clinical and radiological features were consistent with a diagnosis of Erdheim-Chester disease; however, the patient's skin biopsy was consistent with Langerhans cell histiocytosis. PMID:26579323

  17. A Rare Case of Erdheim-Chester Disease and Langerhans Cell Histiocytosis Overlap Syndrome.

    PubMed

    Nabi, Shahzaib; Arshad, Adeel; Jain, Tarun; Virk, Fawad; Gulati, Rohit; Awdish, Rana

    2015-01-01

    A 48-year-old woman with a past medical history of seizures and end-stage renal disease secondary to obstructive uropathy from retroperitoneal fibrosis presented to the emergency department with seizures and altered mental status. A Glasgow Coma Scale of 4 prompted intubation, and she was subsequently admitted to the intensive care unit. Magnetic resonance imaging of the brain performed to elucidate the aetiology of her seizure showed a dural-based mass within the left temporoparietal lobe as well as mass lesions within the orbits. Further imaging showed extensive retroperitoneal fibrosis extending to the mediastinum with involvement of aorta and posterior pleural space. Imaging of the long bones showed bilateral sclerosis and cortical thickening of the diaphyses. Imaging of the maxillofacial structures showed osseous destructive lesions involving the mandible. These clinical and radiological features were consistent with a diagnosis of Erdheim-Chester disease; however, the patient's skin biopsy was consistent with Langerhans cell histiocytosis. PMID:26579323

  18. Langerhans cell histiocytosis with multisystem involvement in an infant: A case report

    PubMed Central

    BI, LINTAO; SUN, BUTONG; LU, ZHENXIA; SHI, ZHANGZHEN; WANG, DAN; ZHU, ZHENXING

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a proliferative disease of histiocyte-like cells, with a wide range of clinical presentations that vary from a solitary lesion to more severe multifocal or disseminated lesions. The disease can affect any age group; however, the peak incidence rate is in infants aged between 1 and 3 years-old. Diagnosis of LCH should be based on the synthetical analysis of clinical presentations, in addition to features of imaging and histopathology. Although certain cases regress spontaneously, other patients require systemic chemotherapy together with the administration of steroids. The present study reports the case of an infant with LDH with multisystem involvement, including that of the bone, skin, orbit, spleen and lungs. The patient received chemotherapy and obtained rapid improvement in the involved systems. A total of 2.5 years after completion of the therapy, the patient still remains in follow-up and no evidence of active disease has been noted. PMID:26136948

  19. Behavioral and hormonal reactivity to threat: Effects of selective amygdala, hippocampal or orbital frontal lesions in monkeys

    PubMed Central

    Machado, Christopher J.; Bachevalier, Jocelyne

    2008-01-01

    Summary We compared the effects of bilateral amygdala, hippocampal or orbital frontal cortex lesions on emotional and hormonal reactivity in rhesus monkeys (Macaca mulatta). Experiment 1 measured behavioral reactivity to an unfamiliar human intruder before and after surgery. Animals with amygdala lesions demonstrated decreases in one passive defensive behavior (freezing), whereas animals with hippocampal lesions showed decreases in a more stimulus-directed defensive behavior (tooth grinding). Orbital frontal cortex lesions also reduced these two defensive behaviors, as well as decreased cage-shaking dominance displays. Animals with amygdala, hippocampal or sham lesions also demonstrated increased tension-related behaviors after surgery, but those with orbital frontal lesions did not. Finally, all three lesions diminished the operated animals' ability to modulate tension-related behaviors depending on the magnitude of threat posed by the human intruder. Experiment 2 measured circulating levels of cortisol and testosterone when a subset of these same animals were at rest and following physical restraint, temporary isolation, exposure to threatening objects and social interactions with an unfamiliar conspecific. None of the lesions impacted on testosterone levels in any condition. Amygdala or orbital frontal lesions blunted cortisol reactivity during isolation from peers, but not during any other condition. Hippocampal lesions did not alter circulating levels of cortisol under any conditions. These results indicate that the amygdala, hippocampus and orbital frontal cortex play distinct, yet complimentary roles in coordinating emotional and hormonal reactivity to threat. PMID:18650022

  20. An unusual case of histiocytosis X in the spine.

    PubMed

    Poulsen, J O; Thommesen, P

    1976-02-01

    A case of spontaneously healing histiocytosis X followed over 9 years is presented. The lesion was located in the cervical and dorsal spine and had unusual radiological features such as soft tissue swelling, involvement of the pedicles and recovery with fusion between adjacent vertebra. PMID:1083620

  1. Lanthanum-Induced Gastrointestinal Histiocytosis

    PubMed Central

    Araya, Hiwot; Longacre, Teri A.; Pasricha, Pankaj J.

    2015-01-01

    A patient with end-stage renal disease (ESRD) on hemodialysis presented with fever, anorexia, and nausea shortly after starting oral lanthanum carbonate for phosphate control. Gastric and duodenal biopsies demonstrated diffuse histiocytosis with intracellular aggregates of basophilic foreign material. Transmission electron microscopy, an underutilized diagnostic test, revealed the nature of the aggregates as heavy metal particles, consistent with lanthanum. Symptoms and histiocytosis improved after discontinuation of lanthanum. Lanthanum may be an underdiagnosed cause of gastrointestinal histiocytosis. PMID:26157959

  2. Langerhans cell histiocytosis: clinical experience with 124 patients.

    PubMed

    Rivera-Luna, R; Martinez-Guerra, G; Altamirano-Alvarez, E; Martinez-Avalos, A; Cardenas-Cardoz, R; Ayon-Cardenas, A; Ruiz-Maldonado, R; Lopez-Corella, E

    1988-08-01

    We cared for 124 pediatric patients with a histologic diagnosis of Langerhans' cell histiocytosis (histiocytosis X) over a period of 14 years. Clinical, laboratory, and radiographic findings were analyzed. The most frequent manifestations were bone lesions, lymph node involvement, and skin infiltration. Liver disease was noted in 50% of patients and lung disease in 23%; hematologic changes were also frequent. Dysfunction and involvement of these three organ systems, plus age of onset, distinguished the group of patients with the highest mortality. All patients with generalized disease or organ dysfunction were treated with systemic chemotherapy. The actuarial survival curve at 10 years was 63%. PMID:3264610

  3. Role of Fine Needle Aspiration Cytology as a Diagnostic Tool in Orbital and Adnexal Lesions

    PubMed Central

    Khan, Lubna; Malukani, Kamal; Malaiya, Siddharth; Yeshwante, Prashant; Ishrat, Saba; Nandedkar, Shirish S.

    2016-01-01

    Purpose: To evaluate the role of fine needle aspiration (FNAC) as a diagnostic tool in cases of orbital and ocular adnexal masses. Cytological findings were correlated with histopathological diagnosis wherever possible. Methods: FNAC was performed in 29 patients of different age groups presenting with orbital and ocular adnexal masses. Patients were evaluated clinically and investigated by non-invasive techniques before fine needle aspiration of the masses. Smears were analyzed by a cytologist in all cases. Further, results of cytology were compared with the histopathological diagnosis. Results: The age of patients ranged from 1 to 68 years (mean: 29.79±19.29). There were 14 males and 15 females with a male to female ratio of 0.93:1. Out of 29 cases, 26 aspirates were cellular. Cellularity was insufficient in three (10.34%) aspirates. Out of 26 cellular aspirates, 11 were non-neoplastic while 15 were neoplastic on cytology. Subsequent histopathologic examination was done in 21/26 cases. Concordance rate of FNAC in orbital and ocular adnexal mass lesions with respect to the precise histologic diagnosis was 90%. Conclusion: When properly used in well-indicated patients (in cases where a diagnosis cannot be made by clinical and imaging findings alone), FNAC of orbital and periorbital lesions is an invaluable and suitable adjunct diagnostic technique that necessitates close cooperation between the ophthalmologist and cytologist. However, nondiagnostic aspirates may sometimes be obtained, and an inconclusive FNAC should not always be ignored. PMID:27621787

  4. A Clinical Update and Radiologic Review of Pediatric Orbital and Ocular Tumors

    PubMed Central

    Rao, Ajay A.; Naheedy, John H.; Chen, James Y.-Y.; Robbins, Shira L.; Ramkumar, Hema L.

    2013-01-01

    While pediatric orbital tumors are most often managed in tertiary care centers, clinicians should be aware of the signs of intraocular and orbital neoplasms. In the pediatric population, a delay in diagnosis of orbital and intraocular lesions, even if benign, can lead to vision loss and deformity. Intraocular lesions reviewed are retinoblastoma, medulloepithelioma, and retinal astrocytic hamartoma. Orbital neoplasms reviewed are rhabdomyosarcoma, neuroblastoma metastases, optic pathway glioma, plexiform neurofibroma, leukemia, lymphoprolipherative disease, orbital inflammatory syndrome, dermoid and epidermoid inclusion cysts, and Langerhans' cell histiocytosis. Vascular lesions reviewed are infantile hemangioma and venous lymphatic malformation. In conjunction with clinical examination, high-resolution ophthalmic imaging and radiologic imaging play an important role in making a diagnosis and differentiating between benign and likely malignant processes. The radiologic imaging characteristics of these lesions will be discussed to facilitate prompt diagnosis and treatment. The current treatment modalities and management of tumors will also be reviewed. PMID:23577029

  5. Otic Langerhans' Cell Histiocytosis in an Adult: A Case Report and Review of the Literature

    PubMed Central

    Gungadeen, Anil; Kullar, Peter; Yates, Philip

    2013-01-01

    Objective. To present a case of otic Langerhans' cell histiocytosis in an adult. Also included the diagnosis and management of the condition and a review of the relevant literature. Case Report. We report a case of a 41-year-old man with a history of persistent unilateral ear discharge associated with an aural polyp. Radiological imaging showed bony lesions of the skull and a soft-tissue mass within the middle ear. Histological analysis of the polyp demonstrated Langerhans' cell histiocytosis. His otological symptoms were completely resolved with the systemic therapy. Conclusions. Otic Langerhans' cell histiocytosis can present in adults. Persistent ear symptoms along with evidence of soft-tissue masses within the ear and bony lesions of the skull or elsewhere should prompt the otolaryngologists to include Langerhans' cell histiocytosis in their differential diagnosis. Management should be with systemic therapy rather than local surgical treatment. PMID:23762704

  6. Progressive Nodular Histiocytosis Associated with Eale's Disease

    PubMed Central

    Williams, Abhilasha; Thomas, Abraham G; Kwatra, Kanwardeep Singh; Jain, Kunal

    2015-01-01

    Progressive nodular histiocytosis (PNH) is a rare normolipemic macrophage disorder and belongs to a subgroup of non-Langerhans cell histiocytosis (LCHs) which is characterized by a progressive course with no sign of spontaneous resolution but without systemic involvement. We report a 30-year-old gentleman who presented with skin lesions all over the body associated with gradual bilateral painless loss of vision. On examination, approximately 30 to 40, skin-colored, firm, non-tender papules and nodules were noted over the body especially on the face and trunk. A skin biopsy revealed a cellular tumor in the dermis composed of oval to spindle-shaped cells, positive for CD68 but negative for S-100, CD34, CD21, CD35 and HMB45, supporting a diagnosis of spindle cell histiocytic tumor. Ophthalmic examination revealed a generalized arteriolar attenuation in both eyes. He received Tab Imatinib 400 mg OD for 5 months followed by Tab Pazopanib 800 mg OD for 4 months and both the drugs were stopped due to lack of any response in the skin lesions. We report this case due to its rarity, characteristic clinical presentation, and its association with Eale's disease. Primary treatment remains surgical excision of bothersome lesions and optimal systemic treatment is still unknown. PMID:26288410

  7. General Information about Langerhans Cell Histiocytosis (LCH)

    MedlinePlus

    ... Langerhans Cell Histiocytosis Treatment (PDQ®)–Patient Version General Information About Langerhans Cell Histiocytosis (LCH) Go to Health ... the PDQ Pediatric Treatment Editorial Board . Clinical Trial Information A clinical trial is a study to answer ...

  8. Langerhans cell histiocytosis: Current concepts in dentistry and case report

    PubMed Central

    Ramos-Gutiérrez, Efraín; Alejo-González, Francisco; Ruiz-Rodríguez, Socorro; Garrocho-Rangel, José-Arturo

    2016-01-01

    Langerhans cell histiocytosis (LCH), which is a rare granulomatous pediatric disease of unknown etiology, is characterized by the idiopathic proliferation and accumulation of abnormal and clonal Langerhans cells or their marrow precursors, resulting in localized, solitary or multiple destructive lesions. These lesions are most commonly eosinophilic granuloma, which are found in craniofacial bone structures such as the skull and mandible, skin and other organs. In children, the disease has a variable initial presentation, and the clinical course, prognosis and survival are unpredictable. The aims of this report were to present an LCH case in a girl aged 2 years, 8 months and her clinicopathological features, to describe the bucodental management provided, and to discuss special dental considerations of this disease. Key words:Children, dental management, histiocytosis, Langerhans cells. PMID:26855698

  9. Late adult onset of Langerhans cell histiocytosis mimicking glioblastoma multiforme.

    PubMed

    Perren, F; Fankhauser, L; Thiévent, B; Pache, J-C; Delavelle, J; Rochat, T; Landis, T; Chizzolini, C

    2011-02-15

    Langerhans cell histiocytosis (LCH) with multiple organ involvement is a rare disorder in adults. Extrapituitary involvement of the central nervous system (CNS) is uncommon. We report the unusual case of a 55-year-old woman presenting with a left-sided hemiataxia-hemiparesis, left hemisensory loss and short-lasting episodes of an alien left hand due to lesions of the internal capsule and the right thalamus, extending into the mesencephalon associated with extensive surrounding edema, without pituitary involvement. The neuroradiological image suggested glioblastoma multiforme. Brain biopsy revealed inflammatory tissue and "pseudotumoral" multiple sclerosis was suspected. Biopsy of concomitant lung and bone lesions disclosed Langerhans cell histiocytosis. The treatment with pulsed steroids in association with mycophenolate mofetil led to a sustained, clinical neurological remission. PMID:21131007

  10. A Retrospective Analysis of Oral Langerhans Cell Histiocytosis in an Iranian Population: a 20-year Evaluation

    PubMed Central

    Atarbashi Moghadam, Saede; Lotfi, Ali; Piroozhashemi, Batool; Mokhtari, Sepideh

    2015-01-01

    Statement of the Problem Langerhans cell histiocytosis is a rare disease with unknown pathogenesis and is characterized by local or disseminated proliferation of Langerhans cells. There is no previous investigation on prevalence of oral Langerhans cell histiocytosis in Iranian population. Purpose The purpose of this study was to assess the relative frequency of oral Langerhans cell histiocytosis in an Iranian population and to compare the data with previous reports. Materials and Method Pathology files of Oral and Maxillofacial Pathology Department of Dental School of Shahid Beheshti University of Medical Sciences from 1992 to 2012 were searched for cases recorded as oral Langerhans cell histiocytosis. A total number of 20 cases were found and the clinical information of patients was recorded. Results The relative frequency of oral Langerhans cell histiocytosis was 0.34% and the most common location was the posterior mandible. In addition, the mean age of patients was 27 years and there was a definite male predominance. Most lesions were localized and tooth mobility was the most common oral presentation. Conclusion In Iranian population as in many other countries, the relative frequency of oral Langerhans cell histiocytosis is low. Moreover, tooth mobility and periodontal lesions are the frequent early signs of disease. Therefore, in patients with periodontal problems, good oral health, and no response to the treatment; Langerhans cell histiocytosis must be considered. Additionally, although most cases of oral Langerhans cell histiocytosis are localized, systemic involvement must also be considered and dental professionals have an important role in early detection of the disease. PMID:26535408

  11. Erdheim–Chester disease associated with intramedullary spinal cord lesion

    PubMed Central

    Takeuchi, T; Sato, M; Sonomura, T; Itakura, T

    2012-01-01

    Erdheim–Chester disease (ECD) is a rare non-Langerhans cell histiocytosis. We present a case of a 56-year-old male with ECD. As time progressed, involvement of the orbital fossa, cranial convexity, spinal cord, brain stem, thyroid, lung, retroperitoneum, lower extremity bones and skin were found. Previously reported cases reveal the frequency of ECD with spinal cord involvement is rare. Although this was a presumed diagnosis based on other lesions, our case is the first in which both intramedullary and epidural masses are present. PMID:22391503

  12. [Pulmonary Langerhans cell histiocytosis].

    PubMed

    Popper, H H

    2015-09-01

    Pulmonary Langerhans cell histiocytosis is regarded as a reactive proliferation of the dendritic Langerhans cell population stimulated by chronic tobacco-derived plant proteins due to incomplete combustion but can also occur in childhood as a tumor-like systemic disease. Currently, both these forms cannot be morphologically distinguished. In the lungs a nodular proliferation of Langerhans cells occurs in the bronchial mucosa and also peripherally in the alveolar septa with an accompanying infiltration by eosinophilic granulocytes and destruction of the bronchial wall. Langerhans cells can be selectively detected with antibodies against CD1a and langerin. In the reactive isolated pulmonary form, abstinence from tobacco smoking in most patients leads to regression of infiltration and improvement of symptoms. In high-resolution computed tomography (HRCT) the small star-like scars can still be detected even after complete cessation of tobacco smoking. PMID:26289803

  13. [Pulmonary Langerhans histiocytosis and Hodgkin's lymphoma].

    PubMed

    Paris, A; Dib, M; Rousselet, M-C; Urban, T; Tazi, A; Gagnadoux, F

    2011-09-01

    Pulmonary Langerhans histiocytosis (PLH) is a rare disease due to the accumulation of Langerhans cells at the level of the bronchioles. These dendritic immunocytes form granulomata and destroy the wall of the airway. We report a case of PLH developing at the same time as Hodgkin's lymphoma in a young woman who smoked tobacco and cannabis. We observed a complete remission of the PLH lesions parallel to the remission of the Hodgkin's lymphoma after chemotherapy, in the absence of any change in the consumption of tobacco and cannabis. This observation leads us to discuss the potential relationships between PLH on one hand, and smoking, the lymphoma and its treatment on the other. PMID:21943541

  14. Cytology of canine malignant histiocytosis.

    PubMed

    Brown, Diane E.; Thrall, Mary Anna; Getzy, David M.; Weiser, M. Glade; Ogilvie, Gregory K.

    1994-01-01

    Cytologic features of bone marrow, tissue, and abdominal fluid in seven cases of malignant histiocytosis in dogs are described, and histopathology, hematology, and serum biochemistry of the cases are reviewed. Diagnosis of malignant histiocytosis was confirmed by tissue morphology and immunohistochemistry; neoplastic cells in all cases had positive immunoreactivity to lysozyme. This stain can be used to definitively establish the diagnosis of malignant histiocytosis on cytology specimens as well as tissue sections. Cytologic findings included numerous pleomorphic, large, discrete mononuclear cells with abundant, lightly basophilic, vacuolated, granular cytoplasm. Nuclei were round to oval to reniform with marked anisocytosis and anisokaryosis; nucleoli were prominent. Mitotic figures, often bizarre, were occasionally seen. Multinucleated giant cells and phagocytosis of erythrocytes and leukocytes were prominent features in cytologic preparations in four cases. Four dogs were anemic, five dogs were thrombocytopenic, and three dogs were hypercalcemic. Breeds affected included Doberman Pinscher (1), Golden Retriever (2), Flat Coated Retriever (3), and mixed-breed dog (1). PMID:12666013

  15. Langerhans cell histiocytosis of the atlas in an adult.

    PubMed

    Zhong, Wo Quan; Jiang, Liang; Ma, Qing Jun; Liu, Zhong Jun; Liu, Xiao Guang; Wei, Feng; Yuan, Hui Shu; Dang, Geng Ting

    2010-01-01

    Langerhans cell histiocytosis (LCH), formerly known as histiocytosis X, is a rare disorder (approximately 1:1,500,000 inhabitants) characterized by clonal proliferation and excess accumulation of pathologic Langerhans cells causing local or systemic effects. The exact etiology of LCH is still unknown. LCH could affect patients of any age, although most present when they are children. The most frequent sites of the bony lesions are the skull, femur, mandible, pelvis and spine. A variety of treatment modalities has been reported, but there was no evidence suggesting that any one treatment was more advantageous than another. We present an adult with LCH of the atlas. A 26-year-old young man presented with a 2-month history of neck pain and stiffness. CT revealed osteolytic lesion in the left lateral mass of atlas with compression fracture. Histopathological diagnosis was Langerhans cell histiocytosis by percutaneous needle biopsy under CT guidance. The patient underwent conservative treatment, including Halo-vest immobilization and radiotherapy. At 7-year follow-up, the patient was asymptomatic except for mild motion restriction of the neck. CT revealed a significant reconstruction of the C1 lateral mass. PMID:19844749

  16. Pulmonary Langerhans cell Histiocytosis.

    PubMed

    Harari, S; Comel, A

    2001-10-01

    Pulmonary Langerhans cell Histiocytosis is a rare granulomatous disease affecting both sexes, with greater incidence in the second and third decades of life; smoking appears to be the most important risk factor. Its etiology is unknown, although there are data indicating an uncontrolled immune response as possible cause, sustained by the Langerhans cells, antigen presenting cells for T lymphocytes, and their accumulation in the distal bronchioles; these cells express on their surface the CD1a and CD1c antigens, and the B7 molecule, essential for activating quiescent T lymphocytes. In its evolution the granuloma is characterized by the progressive reduction in the LC number, with the increase of fibrosis, surrounding and destroying the bronchiolar lumen; the remaining of the lumen, or the traction exerted by fibrous tissue on the adjacent alveolar spaces leads to the cyst development. Vascular involvement occurs frequently, and may explain the onset of pulmonary hypertension in advanced cases of the disease. The disease may be asymptomatic, or it may present with aspecific respiratory signs and symptoms, and has characteristic radiological findings, being included in the group of cysticaerial parenchymal alterations. The diagnosis could be suggested by the finding of a number of LC in BAL greater than 5%. Different therapies have been proposed, but it seems that the most important measure is smoking cessation. PMID:11587096

  17. Langerhans cell histiocytosis of the cervical spine: case report of an unusual location.

    PubMed

    Geusens, E; Brys, P; Ghekiere, J; Samson, I; Sciot, R; Brock, P; Baert, A L

    1998-01-01

    An unusual location for Langerhans cell histiocytosis of the cervical spine is presented. The osteolytic lesion, instead of being located in the vertebral body, was visualised in the left lateral mass of the fifth cervical vertebra, extending into the vertebral body and through the interapophyseal joint into the lateral mass of the fourth cervical vertebra. PMID:9724427

  18. 18F-Fluorodeoxyglucose PET/CT in Langerhans cell histiocytosis: spectrum of manifestations.

    PubMed

    Agarwal, Krishan Kant; Seth, Rachna; Behra, Abhishek; Jana, Manisha; Kumar, Rakesh

    2016-04-01

    The objective of this article is to provide an illustrative tutorial highlighting the utility of 18F-FDG PET/CT imaging to detect the spectrum of manifestations in patients with Langerhans cell histiocytosis. FDG PET/CT is a powerful tool for making an early diagnosis; it allows higher diagnostic confidence with regard to lesions, measuring the extent of disease (staging) and assessing disease activity, and is consequently useful for evaluating the response to therapy in patients with Langerhans cell histiocytosis. PMID:26759026

  19. Langerhans cell histiocytosis with multiple spinal involvement.

    PubMed

    Jiang, Liang; Liu, Xiao Guang; Zhong, Wo Quan; Ma, Qing Jun; Wei, Feng; Yuan, Hui Shu; Dang, Geng Ting; Liu, Zhong Jun

    2011-11-01

    To stress the clinical and radiologic presentation and treatment outcome of Langerhans cell histiocytosis (LCH) with multiple spinal involvements. A total of 42 cases with spinal LCH were reviewed in our hospital and 5 had multifocal spinal lesions. Multiple spinal LCH has been reported in 50 cases in the literature. All cases including ours were analyzed concerning age, sex, clinical and radiologic presentation, therapy and outcome. Of our five cases, three had neurological symptom, four soft tissue involvement and three had posterior arch extension. Compiling data from the eight largest case series of the spinal LCH reveals that 27.2% multiple vertebrae lesions. In these 55 cases, there were 26 female and 29 male with the mean age of 7.4 years (range 0.2-37). A total of 182 vertebrae were involved including 28.0% in the cervical spine, 47.8% in thoracic and 24.2% in the lumbar spine. Extraspinal LCH lesion was documented in 54.2% cases, visceral involvement in 31.1% and vertebra plana in 50% cases. Paravertebral and epidural extension were not documented in most cases. Pathological diagnosis was achieved in 47 cases including 8 open spine biopsy. The treatment strategy varied depending on different hospitals. One patient died, two had recurrence and the others had no evidence of the disease with an average of 7.2 years (range 1-21) of follow-up. Asymptomatic spinal lesions could be simply observed with or without bracing and chemotherapy is justified for multiple lesions. Surgical decompression should be reserved for the uncommon cases in which neurologic compromise does not respond to radiotherapy or progresses too rapidly for radiotherapy. PMID:20496040

  20. Subarachnoid hemorrhage caused by Aspergillus aneurysm as a complication of transcranial biopsy of an orbital apex lesion--case report.

    PubMed

    Okada, Y; Shima, T; Nishida, M; Yamane, K; Yoshida, A

    1998-07-01

    A 62-year-old male complaining of unilateral visual disturbance and pain in the involved eye had a small mass at the right orbital apex which was identified as an Aspergillus granuloma by transcranial biopsy. One month later, the patient became comatose because of fatal subarachnoid hemorrhage due to a newly developed aneurysm. Autopsy showed a ruptured aneurysm on the right internal carotid-posterior communicating artery. Histological examination demonstrated prominent Aspergillus invasion of the arterial wall. Aspergillus infection must be taken into consideration in patients with orbital apex syndrome, which may lead to serious cerebrovascular consequences. If sino-orbital lesions are detected by neuroimaging techniques, biopsy using an extradural approach should be performed to obtain a definitive diagnosis. PMID:9745252

  1. How I treat Langerhans cell histiocytosis.

    PubMed

    Allen, Carl E; Ladisch, Stephan; McClain, Kenneth L

    2015-07-01

    "Langerhans cell histiocytosis" (LCH) describes a spectrum of clinical presentations ranging from a single bone lesion or trivial skin rash to an explosive disseminated disease. Regardless of clinical severity, LCH lesions share the common histology of CD1a(+)/CD207(+) dendritic cells with characteristic morphology among an inflammatory infiltrate. Despite historical uncertainty defining LCH as inflammatory vs neoplastic and incomplete understanding of mechanisms of pathogenesis, clinical outcomes have improved markedly over the past decades through cooperative randomized clinical trials based on empiric therapeutic strategies. Significant advances include recognition of high- and low-risk clinical groups defined by hematopoietic and/or hepatic involvement, and of the importance of optimal intensity and of duration of chemotherapy. Nevertheless, mortality of high-risk patients, disease recurrence, lack of robustly tested salvage strategies, and significant disease morbidity of both high- and low-risk patients remain challenges. Recent discovery of recurrent somatic mutations in mitogen-activated protein kinase pathway genes at critical stages of myeloid hematopoietic differentiation in LCH patients supports redefinition of the disease as a myeloproliferative disorder and provides opportunities to develop novel approaches to diagnosis and therapy. PMID:25827831

  2. Langerhans cell histiocytosis - a case report.

    PubMed

    Jeunon, Thiago; Sousa, Maria Auxiliadora Jeunon; Santos-Rodrigues, Nilton; Lopes, Raquel

    2012-01-01

    A 17-year-old male presented for dermatologic consultation with slightly elevated reddish papules covered by yellowish scales in the scalp for the last two years and reddish and indurated ulcers in the perineum lasting six months. Additional complaints included polyuria, polydipsia, delay in the development of secondary sexual characteristics and hearing loss of the right ear secondary to a medium otitis. Lesions from scalp and perineum were sampled for histopathologic examination and revealed a dense cellular infiltrate made up of mononuclear cells with conspicuous eosinophilic cytoplasm and large cleaved vesicular nucleus, some of them with shapes resembling the format of a kidney and others reminiscent of coffee beans. Numerous intermingling eosinophils were present. The diagnosis of Langerhans cell histiocytosis was then rendered and confirmed by positive immunostaining of neo-plastic cells for anti-CD1a and anti-S100 protein antibodies. The work-up revealed diabetes insipidus, hypogonadotropic hypogonadism, hiperprolactenemia, growing-hormone deficiency and thickness of the pituitary stalk. The patient was treated with prednisone and vinblastin based chemotherapy regimen for six months with complete remission, but presented recurrence of some lesions in the scalp, which were handled with topical mustard and corticosteroids. After chemotherapy, the endocrinologic disturbances were corrected with hormonal replacement therapy. The patient is currently in good health with a follow-up of five years. PMID:24765546

  3. Unifocal Langerhans Cell Histiocytosis Simulating a Limbal Papilloma

    PubMed Central

    Rezaei Kanavi, Mozhgan; Javadi, Fatemeh; Javadi, Mohammad-Ali; Faramarzi, Amir

    2012-01-01

    Purpose To report a rare presentation of unifocal Langerhans cell histiocytosis (LCH) simulating a limbal papilloma. Case report A 24-year-old man presented with a limbal mass in his left eye which had initially been suspected to be a papilloma based on clinical findings. The mass was excised and a histopathological diagnosis of “acute bullous inflammation with granulation tissue” was made. The lesion relapsed 10 months later which necessitated repeat resection along with corneoscleral patch grafting. Histopathological studies of the excised lesion led to a final diagnosis of LCH. Conclusion To the best of our knowledge, this is the second report of a rare presentation of LCH in the limbus which recurred after excision of the primary mass. The recurrent lesion was diagnosed based on histopathology and managed accordingly. PMID:23264867

  4. Langerhans cell histiocytosis revisited: Case report with review

    PubMed Central

    Kumar, Y. Pavan; Agrawal, Jayshree; Mohanlakshmi, J.; Kumar, P. Suresh

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a group of idiopathic disorders characterized by proliferation of bone marrow derived Langerhans cells and mature eosinophils. Their clinical features simulate common oral findings such as gingival enlargement, oral ulcers, and mobility of teeth, along with nonspecific radiographic features; hence, diagnosing such lesions becomes difficult for the oral physicians. These lesions are commonly seen in childhood; however, we are reporting a case of LCH in 29-year-old adult male. A provisional diagnosis of giant cell granuloma was considered based on history and examination, although the lesion was histologically proven to be LCH and was confirmed with immunohistochemical staining of S100 protein and CD1a antigen. The purpose of this paper is to enhance the understanding of diverse, nonpathognomical oral presentation of LCH that is easily misdiagnosed and overlooked by dentist. PMID:26321851

  5. Bisphosphonate Therapy for Refractory Langerhans Cell Histiocytosis: A Case Report.

    PubMed

    Takpradit, Chayamon; Vathana, Nassawee; Narkbunnam, Nattee; Sanpakit, Kleebsabai; Buaboonnam, Jassada

    2015-11-01

    Although patients diagnosed as Langerhans cell histiocytosis (LCH) with bone lesion initially respond well to treatment, some may experience relapse or refractory disease. Pamidronate, a potent N-bisphosphonate, has been used in several primary bone diseases, benign bone tumors, and metastatic bone cancers. The mechanism includes an inhibitory effect on osteoclast activity by decreasing development and recruitment of osteoclast progenitors and promoting osteoclast apoptosis. Herein, we introduce a seven-month-old Thai girl who was diagnosed as multiple-relapse LCH with refractory bone lesions and was treated with standard and salvage steroid-based therapies. After receiving two courses of intravenous pamidronate, she had marked clinical and radiographical improvement without any adverse events. She has been in remission for two years after receiving six courses of therapy. This report supports the efficacy ofpamidronate in LCH-related bone lesions, but further studies in large cohort are warranted. PMID:26817187

  6. Langerhans cell histiocytosis in children diagnosed by fine-needle aspiration

    PubMed Central

    Handa, Uma; Kundu, Reetu; Punia, Rajpal Singh; Mohan, Harsh

    2015-01-01

    Background: Langerhans cell histiocytosis (LCH) is a rare intricate pediatric neoplasm with varied clinical manifestations and multiple treatment modalities. Aim: To study the cytological features of LCH and the differential diagnoses on fine-needle aspiration (FNA). Materials and Methods: FNA was performed using a 23-gauge needle fitted to a 10 mL syringe mounted on syringe holder. LCH was diagnosed on FNA smears in seven cases confined to the head and neck region, which included three cases of lymphadenopathy, three cases of scalp swelling, and one case of orbital swelling. Results: The age of the patients ranged from 25 days to 11 years and male-to-female ratio was 1:1.3. Clinically, the diagnoses suggested were tuberculosis, inflammatory lesion, abscess, and malignancy. The cytologic findings included high cellularity, isolated Langerhans cells (LCs) with prominent nuclear indentation, grooves and abundant vacuolated cytoplasm, multinucleated giant cells, eosinophils, and lymphocytes. Areas of necrosis were noted in one case. Histopathology, along with positive S-100 immunohistochemistry, confirmed the diagnosis of LCH. Conclusions: LCH is a rare disease occurring predominantly in children and can be diagnosed with ease on FNA cytology by the presence of characteristic Langerhans cells. The S-100 positivity aids in suggesting a diagnosis of LCH. PMID:26811572

  7. Langerhans cell histiocytosis: a review of past, current and future therapies.

    PubMed

    Allen, Carl E; McClain, Kenneth L

    2007-09-01

    The goal of therapy in Langerhans cell histiocytosis (LCH) is to decrease the activity and proliferation of histiocytes, lymphocytes and macrophages that cause the disease. Patients with disease that is localized to skin, bone and lymph node (defined as "nonrisk" organs) generally have a good prognosis and require minimal treatment. However, patients with lesions in "risk" organs (liver, spleen, lung, bone marrow) have a worse overall prognosis regarding mortality and morbidity. Likewise, patients with LCH in the central nervous system (CNS), vertebrae, facial bones or bones of the anterior or middle cranial fossa are at higher risk for morbidity and recurrent disease. LCH in the orbit, mastoid or temporal skull regions are classified as "CNS risk" because of an increased frequency of developing diabetes insipidus and other endocrine abnormalities or parenchymal brain lesions. Outcomes of patients with LCH in only one bone in "nonrisk" locations are generally benign, and the disease responds well to several treatment modalities including observation, surgical excision, steroid injection or radiation therapy. The last is generally reserved for a single vertebral lesion or when a risk of pathologic fracture exists in the greater trochanter. The greatest challenge in treatment of LCH is patients with multisystem disease. Patients with persistent or worsening disease in risk organs by the end of the first 6-12 weeks of therapy have significantly decreased overall survival regardless of treatment. Additionally, optimal treatment for patients with late-onset CNS symptoms and adults with LCH remain to be defined. In this article we will review the evolution of multicenter and international treatment studies as well as the current Histiocyte Society research treatment protocol, LCH-III. We also review experiences with a variety of agents that have been used to treat LCH outside of clinical trials. Since LCH is a rare disease in children and adults, these patients should be

  8. Genetics Home Reference: Langerhans cell histiocytosis

    MedlinePlus

    ... cell histiocytosis affects the lungs, liver, or blood-forming (hematopoietic) system; damage to these organs and tissues ... occurs when the Langerhans cells crowd out blood-forming cells in the bone marrow, leads to a ...

  9. Langerhans cell histiocytosis: a cytokine/chemokine-mediated disorder?

    PubMed

    Garabedian, Lara; Struyf, Sofie; Opdenakker, Ghislain; Sozzani, Silvano; Van Damme, Jo; Laureys, Geneviève

    2011-09-01

    Langerhans cell histiocytosis (LCH) is a rare disorder characterized by an abnormal accumulation and/or proliferation of cells with a Langerhans cell phenotype. Although no clear cause of LCH has been identified, it has been postulated that LCH might be the consequence of an immune dysregulation, causing Langerhans cells to migrate to and accumulate at various sites. Production of cytokines and chemokines is a central feature of immune regulation. Cytokines are abundantly present within LCH lesions. We review here the potential role of cytokines and chemokines in the pathogenesis of LCH. The type, distribution, and number of different cytokines released within lesions can provide clues to the possible aetiology of LCH and, ultimately, might offer therapeutic possibilities using recombinant cytokines or antagonists for this disorder. PMID:22001902

  10. Efficacy of High Frequency Ultrasound in Localization and Characterization of Orbital Lesions

    PubMed Central

    Gurushankar, G; Bhimarao; Kadakola, Bindushree

    2015-01-01

    Background The complicated anatomy of orbit and the wide spectrum of pathological conditions present a formidable challenge for early diagnosis, which is critical for management. Ultrasonography provides a detailed cross sectional anatomy of the entire globe with excellent topographic visualization and real time display of the moving organ. Objectives of the study To evaluate the efficacy of high frequency Ultrasound in localization of orbital diseases and to characterize various orbital pathologies sonologically. Materials and Methods Hundred eyes of 85 patients were examined with ultrasound using linear high frequency probe (5 to 17 MHz) of PHILPS IU22 ultrasound system. Sonological diagnosis was made based on location, acoustic characteristics, kinetic properties and Doppler flow dynamics. Final diagnosis was made based on clinical & laboratory findings/higher cross-sectional imaging/surgery & histopathology (as applicable). Diagnostic accuracy of ultrasonography was evaluated and compared with final diagnosis. Results The distinction between ocular and extraocular pathologies was made in 100% of cases. The overall sensitivity, specificity, NPV and accuracy of ultrasonography were 94.2%, 98.8%, 92.2% & 94.9% respectively for diagnosis of ocular pathologies and 94.2%, 99.2%, 95.9% & 95.2% respectively for extra ocular pathologies. Conclusion Ultrasonography is a readily available, simple, cost effective, non ionizing and non invasive modality with overall high diagnostic accuracy in localising and characterising orbital pathologies. It has higher spatial and temporal resolution compared to CT/MRI. However, CT/MRI may be indicated in certain cases for the evaluation of calcifications, bony involvement, extension to adjacent structures and intracranial extension. PMID:26500977

  11. False-positive PET/CT for lymphoma recurrence secondary to Langerhans cell histiocytosis.

    PubMed

    Fotos, Joseph S; Flemming, Donald J; Tulchinsky, Mark

    2011-08-01

    A 28-year-old man with a history of Hodgkin lymphoma that was intensely [F-18]fluoro-2-deoxy-D-glucose-avid at diagnosis had achieved complete remission following appropriate therapy. On surveillance positron emission tomography/computed tomography (CT), new, intensely [F-18]fluoro-2-deoxy-D-glucose-avid lesions (lytic on CT) were seen within the vertebral body of C7, left scapula, and right glenoid. The findings of a biopsy revealed these lesions to be Langerhans cell histiocytosis. Langerhans cell histiocytosis arising in the context of lymphoma is a well-documented phenomenon, and its appearance on positron emission tomography/CT cannot be conclusively distinguished from lymphoma recurrence. This manuscript emphasizes the necessity of biopsy to ensure correct diagnosis and subsequent correct therapy. PMID:21716031

  12. [Langerhans cell histiocytosis in adults].

    PubMed

    Néel, A; Artifoni, M; Donadieu, J; Lorillon, G; Hamidou, M; Tazi, A

    2015-10-01

    Langerhans cell histiocytosis (LCH) is a rare disease characterized by the infiltration of one or more organs by Langerhans cell-like dendritic cells, most often organized in granulomas. The disease has been initially described in children. The clinical picture of LCH is highly variable. Bone, skin, pituitary gland, lung, central nervous system, lymphoid organs are the main organs involved whereas liver and intestinal tract localizations are less frequently encountered. LCH course ranges from a fulminant multisystem disease to spontaneous resolution. Several randomized controlled trials have enable pediatricians to refine the management of children with LCH. Adult LCH has some specific features and poses distinct therapeutic challenges, knowing that data on these patients are limited. Herein, we will provide an overview of current knowledge regarding adult LCH and its management. We will also discuss recent advances in the understanding of the disease, (i.e. the role of BRAF oncogene) that opens the way toward targeted therapies. PMID:26150351

  13. Langerhans cell histiocytosis in adults: a case report and review of the literature

    PubMed Central

    2016-01-01

    Background Langerhans cell histiocytosis (LCH) is a proliferative disease of histiocyte-like cells that generally affects children. Immunohistochemistry is essential to obtain the correct diagnosis, and treatment protocols are controversial. Objective Langerhans cell histiocytosis (LCH) is easy to be misdiagnosed because of its various clinic features and laboratory results. This research focused on the clinicopathological, histopathological, immunohistochemical and other features of LCH and aimed to analyze LCH clinical features for improving diagnosis and decreasing misdiagnosis rate. Case report A case of rare adult LCH was reported and the clinicopathological features were summarized by literature review. The multifocal form of this case includes diabetes insipidus, exophthalmos and mucocutaneous lesions in axillae and anogenital regions, such as infiltrated nodules, extensive coalescing, scaling, crusted papules and ulcerated plaques. The Langerhans cells diffusely infiltrated in the dermis and the tumor cells were positive for CD1a and S-100 expression. The diagnosis was Langerhans cell histiocytosis based on the pathological and immunohistochemical changes. Conclusion LCH has high rate of misdiagnosis and definitive diagnosis depends on pathological biopsy and X-ray examination. The prognosis is related to the onset age and the quantity of affected organs. Although specific therapeutic approach hasn't been well established, combined chemotherapy for multisystem lesions and surgical operation or radiotherapy for unifocal lesions may improve the therapy. PMID:26942568

  14. Recurrent NRAS mutations in pulmonary Langerhans cell histiocytosis.

    PubMed

    Mourah, Samia; How-Kit, Alexandre; Meignin, Véronique; Gossot, Dominique; Lorillon, Gwenaël; Bugnet, Emmanuelle; Mauger, Florence; Lebbe, Celeste; Chevret, Sylvie; Tost, Jörg; Tazi, Abdellatif

    2016-06-01

    The mitogen-activated protein kinase (MAPK) pathway is constantly activated in Langerhans cell histiocytosis (LCH). Mutations of the downstream kinases BRAF and MAP2K1 mediate this activation in a subset of LCH lesions. In this study, we attempted to identify other mutations which may explain the MAPK activation in nonmutated BRAF and MAP2K1 LCH lesions.We analysed 26 pulmonary and 37 nonpulmonary LCH lesions for the presence of BRAF, MAP2K1, NRAS and KRAS mutations. Grossly normal lung tissue from 10 smoker patients was used as control. Patient spontaneous outcomes were concurrently assessed.BRAF(V600E) mutations were observed in 50% and 38% of the pulmonary and nonpulmonary LCH lesions, respectively. 40% of pulmonary LCH lesions harboured NRAS(Q61K) (/R) mutations, whereas no NRAS mutations were identified in nonpulmonary LCH biopsies or in lung tissue control. In seven out of 11 NRAS(Q61K) (/R)-mutated pulmonary LCH lesions, BRAF(V600) (E) mutations were also present. Separately genotyping each CD1a-positive area from the same pulmonary LCH lesion demonstrated that these concurrent BRAF and NRAS mutations were carried by different cell clones. NRAS(Q61K) (/R) mutations activated both the MAPK and AKT (protein kinase B) pathways. In the univariate analysis, the presence of concurrent BRAF(V600E) and NRAS(Q61K) (/R) mutations was significantly associated with patient outcome.These findings highlight the importance of NRAS genotyping of pulmonary LCH lesions because the use of BRAF inhibitors in this context may lead to paradoxical disease progression. These patients might benefit from MAPK kinase inhibitor-based treatments. PMID:27076591

  15. Bilateral Temporal Bone Langerhans Cell Histiocytosis: Radiologic Pearls

    PubMed Central

    Coleman, Mira A.; Matsumoto, Jane; Carr, Carrie M.; Eckel, Laurence J.; Nageswara Rao, Amulya A.

    2013-01-01

    Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder with an unpredictable clinical course and highly varied clinical presentation ranging from single system to multisystem involvement. Although head and neck involvement is common in LCH, isolated bilateral temporal bone involvement is exceedingly rare. Furthermore, LCH is commonly misinterpreted as mastoiditis, otitis media and otitis externa, delaying diagnosis and appropriate therapeutic management. To improve detection and time to treatment, it is imperative to have LCH in the differential diagnosis for unusual presentations of the aforementioned infectious head and neck etiologies. Any lytic lesion of the temporal bone identified by radiology should raise suspicion for LCH. We hereby describe the radiologic findings of a case of bilateral temporal bone LCH, originally misdiagnosed as mastoiditis. PMID:24478812

  16. Adult onset folliculocentric langerhans cell histiocytosis confined to the scalp.

    PubMed

    Hancox, John G; James, Asha Pardasani; Madden, Christopher; Wallace, Christopher A; McMichael, Amy J

    2004-04-01

    Langerhans cell histiocytosis (LCH) is a pleomorphic disease entity characterized by local or disseminated atypical Langerhans cells found most commonly in bone, lungs, mucocutaneous structures, and endocrine organs. Cutaneous disease occurs in approximately one quarter of all cases. Cutaneous findings include soft-tissue swelling, eczematous changes, a seborrheic dermatitis-like appearance, and ulceration. We report a rare case of LCH confined to the scalp with folliculocentric infiltrates. This 32-year-old male patient presented with follicularly based erythema, scale, and pustules unresponsive to topicals and oral antibiotics. The patient's lesions mimicked lichen planopilaris and folliculitis decalvans during the disease process. On hematoxylin and eosin stain, scalp biopsy showed a perivascular interstitial patchy lichenoid mononuclear cell infiltrate that focally abutted follicular infundibula. Prominent mononuclear cells having reniform nuclei were present, and immunoperoxidase stains for CD1a confirmed Langerhans cell differentiation. Serological and imaging workup failed to display systemic involvement. PMID:15024194

  17. Bisphosphonates in Langerhans Cell Histiocytosis: An International Retrospective Case Series

    PubMed Central

    Chellapandian, Deepak; Makras, Polyzois; Kaltsas, Gregory; van den Bos, Cor; Naccache, Lamia; Rampal, Raajit; Carret, Anne-Sophie; Weitzman, Sheila; Egeler, R. Maarten; Abla, Oussama

    2016-01-01

    Background Bone is the most common organ of involvement in patients with Langerhans cell histiocytosis (LCH), which is often painful and associated with significant morbidity from pathological fractures. Current first-line treatments include chemotherapy and steroids that are effective but often associated with adverse effects, whereas the disease may reactivate despite an initial response to first-line agents. Bisphosphonates are osteoclast inhibitors that have shown to be helpful in treating bone lesions of LCH. To date, there are no large international studies to describe their role in treating bone lesions of LCH. Method We conducted a multicenter retrospective review of 13 patients with histologically proven LCH, who had received bisphosphonates either at diagnosis or at disease reactivation. Results Ten patients (77%) had a single system bone disease, and 3 (23%) had bone lesions as part of multisystem disease. Median follow-up time post-bisphosphonate therapy was 4.6 years (range, 0.8 to 8.2 years). Treatment with bisphosphonates was associated with significant pain relief in almost all patients. Twelve (92%) achieved resolution of active bone lesions, and 10 out of them had no active disease for a median of 3.5 years (range, 0.8 to 5 years). One patient did not respond. No major adverse effects were reported in this series. Conclusion Bisphosphonates are well-tolerated drugs that can significantly improve bone pain and induce remission in active bone LCH. Future prospective studies evaluating the role of bisphosphonates in LCH are warranted. PMID:27413525

  18. Langerhans cell histiocytosis followed by Hodgkin's lymphoma.

    PubMed

    Park, Ik Soo; Park, In Keun; Kim, Eun Kyoung; Kim, Shin; Jeon, Sang Ryong; Huh, Joo Ryung; Suh, Cheol Won

    2012-12-01

    A 22-year-old man was referred to our institution due to lower back pain and was diagnosed with Langerhans cell histiocytosis of the thoracic and lumbar spine. The patient achieved complete remission with radiotherapy and chemotherapy. One year later, right cervical lymphadenopathy was observed and Hodgkin's lymphoma was confirmed on biopsy. The patient was treated with chemotherapy and autologous stem cell transplantation, and experienced no further symptoms. Further, no evidence of recurrence was observed on follow-up imaging. This report discusses the association between Langerhans cell histiocytosis and Hodgkin's lymphoma. PMID:23269889

  19. Langerhans Cell Histiocytosis Followed by Hodgkin's Lymphoma

    PubMed Central

    Park, IK Soo; Park, In Keun; Kim, Eun Kyoung; Kim, Shin; Jeon, Sang Ryong; Huh, Joo Ryung

    2012-01-01

    A 22-year-old man was referred to our institution due to lower back pain and was diagnosed with Langerhans cell histiocytosis of the thoracic and lumbar spine. The patient achieved complete remission with radiotherapy and chemotherapy. One year later, right cervical lymphadenopathy was observed and Hodgkin's lymphoma was confirmed on biopsy. The patient was treated with chemotherapy and autologous stem cell transplantation, and experienced no further symptoms. Further, no evidence of recurrence was observed on follow-up imaging. This report discusses the association between Langerhans cell histiocytosis and Hodgkin's lymphoma. PMID:23269889

  20. Rare C-6 vertebral involvement in a child with histiocytosis X: case report.

    PubMed

    Salehpour, Firouz; Tubbs, R Shane; Zarrintan, Sina; Meshkini, Ali; Hadidchi, Shahram; Pourfathi, Hojjat; Azhough, Ramin; Fakhrjou, Asharf; Goodrich, James T; Khaki, Amir Afshin

    2007-01-01

    The authors present a rare case of C-6 vertebral involvement in a 12-year-old boy with histiocytosis X. The patient presented with limitation in movements of neck and upper extremities. Computed tomography (CT) and magnetic resonance (MRI) imaging were used in the preoperative workup. Surgery was performed via an anterior cervical approach along with stabilization using a fibula strut graft and plate fixation. Pathological assessment showed infiltration of Langerhans cells accompanied by a mixture of many eosinophils, giant cells, neutrophils and foamy cells. The patient went on to make a full recovery with complete resolution of his motor weakness. In an extensive review of the literature there are very few cases of cervical histiocytosis X reported. In addition, surgical management of this type of lesion has rarely been discussed. PMID:17594600

  1. Langerhans cell histiocytosis of the digestive tract identified on an upper gastrointestinal examination.

    PubMed

    Zei, Markus; Meyers, Arthur B; Boyd, Kevin P; Larson-Nath, Catherine; Suchi, Mariko

    2016-08-01

    Langerhans cell histiocytosis (LCH) with involvement of the gastrointestinal tract is rare and typically identified in patients with systemic disease. We describe a 16-month-old girl who initially presented with bilious vomiting, failure to thrive and a rash. An upper gastrointestinal (GI) examination revealed loss of normal mucosal fold pattern and luminal narrowing within the duodenum, prompting endoscopic biopsy. Langerhans cell histiocytosis of the digestive tract was confirmed by histopathology. A skeletal survey and skin biopsy identified other systemic lesions. Although uncommon, it is important to consider LCH in the differential diagnosis for gastrointestinal symptoms of unclear origin, especially when seen with concurrent rash. Findings of gastrointestinal involvement on upper GI examination include loss of normal mucosal fold pattern and luminal narrowing in the few published case reports. PMID:26886914

  2. [Neuroimaging of Langerhans cell histiocytosis in the central nervous system of children].

    PubMed

    De La Hoz Polo, M; Rebollo Polo, M; Fons Estupiña, C; Muchart López, J; Cruz Martinez, O

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare disease characterized by the accumulation within tissues of anomalous dendritic cells similar to Langerhans cells. The clinical presentation varies, ranging from the appearance of a single bone lesion to multisystemic involvement. Central nervous system (CNS) involvement, manifesting as diabetes insipidus secondary to pituitary involvement, has been known since the original description of the disease. Two types of CNS lesions are currently differentiated. The first, pseudotumoral lesions with infiltration by Langerhans cells, most commonly manifests as pituitary infiltration. The second, described more recently, consists of neurodegenerative lesions of the CNS associated with neurologic deterioration. This second type of lesion constitutes a complication of the disease; however, there is no consensus about the cause of this complication. Our objective was to describe the radiologic manifestations of LCH in the CNS in pediatric patients. PMID:24837565

  3. Langerhans cell histiocytosis of the sacrum

    PubMed Central

    Hatem, M.A.

    2015-01-01

    Langerhans cell histiocytosis is a rare disease with a wide spectrum of clinical presentations. It is a multisystemic disease with organ system involvement ranging from simple—where it involves only one organ—to widespread progressive disease. Although it can affect any age group, the peak incidence is between 1 and 3 years of age.

  4. [Vertebral changes in histiocytosis x (author's transl)].

    PubMed

    Greinacher, I; Gutjahr, P

    1978-06-01

    Manifestations of histiocytosis X in the vertebral column occurred in 3 of 15 children. Pathological alterations showed a marked variability. Especially a vertebra plana should be considered as eosinophilic granuloma, until another cause is proven. Bone scans were positive in all three cases. Even in most severe cases with vertebral destruction neurological abnormalities were absent. PMID:308238

  5. Conjunctival langerhans cell histiocytosis: a case report.

    PubMed

    Chen, Di; Min, Han-Yi

    2015-03-01

    Langerhans cell histiocytosis (LCH) is a rare disease, mainly involving the bone, skin, lung, liver, spleen, and skin.1 The heterogenenous nature of LCH makes it difficult to diagnose. Not only do the involved organs vary from case to case, but also its natural history. Herein, we describe a rare case of conjuctival LCH in an Asian woman. PMID:25837364

  6. Severe Periodontal Disease Manifested in Chronic Disseminated Type of Langerhans Cell Histiocytosis in a 3-Year Old Child

    PubMed Central

    Srivastava, Vinay Kumar; Bansal, Rajesh; Gupta, Vineeta; Bansal, Manish; Patne, Shashikant

    2014-01-01

    ABSTRACT% Langerhans cell histiocytosis (LCH), previously known as histio-cytosis X, is a rare idiopathic disorder of reticulo-endothelial system with abnormal proliferation of bone marrow derived Langerhans cells along with a variable number of leukocytes, such as eosinophils, neutrophils, lymphocytes and plasma cells. Three years old male child presented with multifocal osteolytic lesions and papulosquamous skin lesions. Clinical and radio-graphic features, such as severe alveolar bone loss, mobility of teeth, precocious eruption of teeth, foating appearance of teeth in orthopantomogram (OPG), osteolytic lesion in skull and cutaneous lesions were highly suggestive of LCH disease. Skin biopsy confirmed a diagnosis of LCH. Induction chemotherapy with oral prednisolone and intravenous vinblastine was started. Child responded well to chemotherapy. The clinical significance of the presented case is to diagnose the case of LCH on the basis of the manifestation of severe periodontal disease as this can be first or only manifestation of LCH. A dentist plays a major role in the multidisciplinary treatment of LCH through routine examination and periodic follow-up. How to cite this article: Bansal M, Srivastava VK, Bansal R, Gupta V, Bansal M, Patne S. Severe Periodontal Disease Manifested in Chronic Disseminated Type of Langerhans Cell Histiocytosis in a 3-Year Old Child. Int J Clin Pediatr Dent 2014;7(3):217-219. PMID:25709306

  7. A rare presentation of langerhans cell histiocytosis tonsil infiltration: review of the literature: atypical presentation of langerhans cell histiocytosis.

    PubMed

    Atalay, Figen; Koç, Eltaf Ayça Özbal; Yıldız, Semsi

    2014-09-01

    Langerhans cell histiocytosis (LCH) is a rare disease that can infiltrate various organs. LCH presents with solitary organ involvement or as a multi-system disease. We present a patient who has tonsillary infiltration with LCH. A 74 year-old Caucasian male was admitted for swelling of the neck and difficulty swallowing for 3 months. Physical examination showed submandibular lymph node enlargement of approximately 3 cm and tonsil enlargement. A tonsillectomy and excisional biopsy of the lymph node were done. Histiocyte-like cell infiltration was seen in the tonsil biopsy. CD3, CD20, CD15, CD30, CD5, CD138, Lambda, Kappa, Bcl-2, ALK, CD23, CD10, Bcl-6, keratin, EMA, HMB-45, and Cyl D1 were negative. CD68, S-100, CD1a, and fascin were positive, and the Ki-67 proliferation index was 20 % in immunocytochemical staining. The most commonly infiltrated bones are the skull, femur, lower jaw, pelvis, and vertebrae in LCH. Oral or perioral lesions are present in 30 % of cases. Oral lesions most often involve bone loss, unexpected tooth loss, and gum inflammation. We administered oral prednisolone to our patient due to the presence of lytic lesion of the bone, mild anemia and a higher sedimentation rate, which was from a separate, explained cause. Isolated tonsillar involvement in adult LCH was reported in only 2 cases in the literature. There is no standard recommendation for treatment. Our patient responded well to steroid therapy. PMID:25332640

  8. Langerhans cell histiocytosis of the thyroid with multiple cervical lymph node involvement accompanying metastatic thyroid papillary carcinoma.

    PubMed

    Ceyran, A Bahar; Senol, Serkan; Bayraktar, Barış; Ozkanlı, Seyma; Cinel, Z Leyla; Aydın, Abdullah

    2014-01-01

    A 37-year-old male case was admitted with goiter. Ultrasonography of thyroid showed a 5 cm cystic nodule in the left lobe with a 1.5 cm solid component. Fine needle aspiration biopsy revealed atypia of undetermined significance or follicular lesion. The patient was operated on. The pathological diagnosis was reported as papillary thyroid carcinoma. The immunohistochemical examination showed multiple foci of Langerhans cell histiocytosis involving both lobes. The patient died due to cardiac arrest with respiratory causes in the early postoperative period. Langerhans cell histiocytosis is a rare primary condition which involves abnormal clonal proliferation of Langerhans cells in various tissues and organs. Thyroid involvement is infrequently seen. Although the etiology is unknown, genetic components may be linked to the disease. It is also associated with a family history of thyroid disease. Papillary thyroid carcinoma is the most common malignant epithelial tumor of the thyroid gland. Langerhans cell histiocytosis presenting with papillary thyroid carcinoma is rare. The privilege of our case is langerhans cell histiocytosis of the thyroid with multiple cervical lymph node involvement accompanying cervical lymph node metastatic thyroid papillary carcinoma. PMID:25349760

  9. Langerhans Cell Histiocytosis of the Thyroid with Multiple Cervical Lymph Node Involvement Accompanying Metastatic Thyroid Papillary Carcinoma

    PubMed Central

    Ceyran, A. Bahar; Şenol, Serkan; Bayraktar, Barış; Özkanlı, Şeyma; Cinel, Z. Leyla; Aydın, Abdullah

    2014-01-01

    A 37-year-old male case was admitted with goiter. Ultrasonography of thyroid showed a 5 cm cystic nodule in the left lobe with a 1.5 cm solid component. Fine needle aspiration biopsy revealed atypia of undetermined significance or follicular lesion. The patient was operated on. The pathological diagnosis was reported as papillary thyroid carcinoma. The immunohistochemical examination showed multiple foci of Langerhans cell histiocytosis involving both lobes. The patient died due to cardiac arrest with respiratory causes in the early postoperative period. Langerhans cell histiocytosis is a rare primary condition which involves abnormal clonal proliferation of Langerhans cells in various tissues and organs. Thyroid involvement is infrequently seen. Although the etiology is unknown, genetic components may be linked to the disease. It is also associated with a family history of thyroid disease. Papillary thyroid carcinoma is the most common malignant epithelial tumor of the thyroid gland. Langerhans cell histiocytosis presenting with papillary thyroid carcinoma is rare. The privilege of our case is langerhans cell histiocytosis of the thyroid with multiple cervical lymph node involvement accompanying cervical lymph node metastatic thyroid papillary carcinoma. PMID:25349760

  10. Langerhans Cell Histiocytosis of the Clavicle in an Adult: A Case Report and Review of the Literature

    PubMed Central

    Udaka, Toru; Susa, Michiro; Kikuta, Kazutaka; Nishimoto, Kazumasa; Horiuchi, Keisuke; Sasaki, Aya; Kameyama, Kaori; Nakamura, Masaya; Matsumoto, Morio; Chiba, Kazuhiro; Morioka, Hideo

    2015-01-01

    Langerhans cell histiocytosis (LCH) usually occurs in children under the age of 10 years with a predilection for the skull, spine, rib and humerus. Solitary LCH occurring in an adult clavicle is uncommon with limited reports to date. The lesion in our patient was curetted with the intent to make a diagnosis, which subsequently lead to the remission of the symptom and the disease. At the final follow-up after 1 year, no local recurrence or metastasis is observed. PMID:26600774

  11. Diagnosis and Intralesional Corticotherapy in Oral Ulcers Occurring as the Sole Manifestation of Langerhans Cell Histiocytosis. A Case Report

    PubMed Central

    Gambirazi, Liane; Libório, Tatiana; Nunes, Fábio; Sugaya, Norberto; Migliari, Dante

    2016-01-01

    This article reports a case of oral mucosa lesions as the sole manifestation in Langerhans cell histiocytosis (LCH). This is a very uncommon manifestation of LCH since this disease preferably affects the bones with frequent involvement of the jaws. LCH may also involve other organs, particularly the lungs, liver, lymph nodes, and skin. The highlights of this report are the differential diagnosis, immunohistochemical analysis and, mostly, the therapeutic approach. PMID:27398106

  12. Unifocal Granuloma of Femur due to Langerhans' Cell Histiocytosis: A Case Report and Review of the Literature

    PubMed Central

    Singh, Harpreet; Kaur, Satnam; Yuvarajan, P.; Jain, Nishant; Maini, Lalit

    2010-01-01

    The radiological diagnosis of osteolytic lesions of the long bones in pediatric population constitutes a challenge when the case history and clinical data are uncharacteristic. We believe that the description of few clinically and histologically proven cases to verify the existence of radiological signs useful for diagnosis may be of interest. Here, we describe a case of Langerhans' cell histiocytosis (LCH) presenting as unifocal eosinophilic granuloma of femur along with a brief review of the literature. PMID:20811571

  13. Langerhans cell histiocytosis case with dense metaphyseal band sign.

    PubMed

    Kikkawa, Ichiro; Aihara, Toshinori; Morimoto, Akira; Watanabe, Hideaki; Furukawa, Rieko

    2013-02-01

    Eosinophilic granuloma, a type of Langerhans cell histiocytosis, exhibits a classic vertebral collapse, which is called vertebra plana (Calve's disease) and it manifests as a solitary bony lesion. Vertebra plana can cause severe pain in patients. Bisphosphonates (clodronate, pamidronate and zoledronic acid) have been recently used to treat osteolytic bone lesions of LCH. Zoledronic acid has 100 times relative potency that of pamidronate. We report a case of a 10-year-old girl who had zoledronic acid treatment for severe back pain due to vertebra plana. X-ray photographs of the patient's body showed dense metaphyseal band sign, which can be found in lead poisoning, treated leukemia, healing rickets, recovery from scurvy, vitamin D hypervitaminosis, congenital hypothyroidism and hypoparathyroidism. Increased biological potent zoledronic acid deprived her of severe back pain due to vertebra plana and might cause dense metaphyseal band sign of her skeleton. Conclusion; We have cured the severe back pain of a 10-year-old girl case of eosinophilic granuloma with zoledronic acid. After that treatment, X-ray photographs of the patient's body showed dense metaphyseal band sign. There have been few such cases reported until now. PMID:23409985

  14. [Secondary biliary cirrhosis in a patient with histiocytosis X].

    PubMed

    Romão, Zita; Pontes, J; Andrade, P; Leitão, M C; Donato, A; Freitas, Diniz

    2002-01-01

    Histiocitose X or Histiocytosis of the Langerhans cells represents a complex spectrum of clinical alterations, resulting from infiltration by anomalous histiocytes of various organs, including the skin, bones, lungs, lymphatic ganglia and liver. Liver disease is rare and the mechanism by which lesions appear is unknown. Cholestasis results from phenomena of sclerosant colangitis, which affects the intrahepatic ducts, or from proliferation of histiocytic cells in the periportal areas. Some patients develop biliar cyrrhosis. The authors present the clinical case of a 62-year-old female patient, hospitalized for chronic cholestasis, diabetes and gallstone in the main bile duct. She had metastatic lesions of the hypophysis and bones, the biopsies of which revealed infiltration by histiocytic cells. Endoscopic Retrograde Colangiopancreatography (E.R.C.P.) revealed dilatation of intrahepatic bile ducts and stenosis of left hepatic duct. A histological study of the hepatic biopsy showed chronic cholestasis and areas of fibrosis, without infiltration by histiocytic granulomas, which were observed in the medular biopsy and in the thyroid nodule cytology. PMID:12025455

  15. New Insights Into the Molecular Pathogenesis of Langerhans Cell Histiocytosis

    PubMed Central

    Rizzo, Francesca M.; Cives, Mauro; Simone, Valeria

    2014-01-01

    Langerhans cell histiocytosis (LCH) is a rare proliferative disorder characterized by an accumulation of cells sharing the major phenotypic features of cutaneous Langerhans cells. Given its variable clinical evolution, ranging from self-limiting lesions to multisystemic forms with a poor prognosis, in the last decades it has been debated whether LCH might not have a neoplastic rather than an inflammatory nature. However, although the fundamental events underlying the pathogenesis of LCH are still elusive, recent advances have strikingly improved our understanding of the disease. In particular, the identification of multiple interplays between LCH cells and their tumor microenvironment, along with the recognition of the lesional cytokine storm as a key determinant of LCH progression, has substantiated new opportunities for devising targeted therapeutic approaches. Strikingly, the detection of the rapidly accelerated fibrosarcoma isoform BV600E gain-of-function mutation as a genetic alteration recurring in more than 50% of patients has fueled the paradoxical picture of LCH as a tumor of the antigen-presenting cells that can evade rejection by the immune system. Thus, new evidence regarding the ontogeny of LCH cells, as well as a better understanding of the putative immune system frustrating strategy in LCH, may help to define the precise pathogenesis. PMID:24436311

  16. Indeterminate cell histiocytosis successfully treated with phototherapy.

    PubMed

    Zerbini, Maria Claudia Nogueira; Sotto, Mirian Nacagami; de Campos, Fernando Peixoto Ferraz; Abdo, Andre Neder Ramires; Pereira, Juliana; Sanches, José Antônio; Martins, Jade Cury

    2016-01-01

    First described in 1985, intermediate cell histiocytosis is a rare disorder of the cutaneous dendritic cell group with a varied clinical presentation and evolution. The pathologic substrate is constituted by the proliferation of indeterminate cells (ICs) that are immunophenotypically characterized by the positivity of CD1a, CD68, and faint/focal S100, plus the negativity for CD207 (langerin). The authors present the case of a healthy elderly woman who presented generalized dome-shaped reddish cutaneous nodules over her trunk, neck, face, and extremities over a period of 18 months. A laboratory and imaging work-up ruled out internal involvement. The skin biopsy was consistent with IC histiocytosis. The patient was treated with narrowband ultraviolet B phototherapy, which resulted in an excellent short-term outcome. PMID:27547741

  17. Indeterminate cell histiocytosis successfully treated with phototherapy

    PubMed Central

    Sotto, Mirian Nacagami; de Campos, Fernando Peixoto Ferraz; Abdo, Andre Neder Ramires; Pereira, Juliana; Sanches, José Antônio; Martins, Jade Cury

    2016-01-01

    First described in 1985, intermediate cell histiocytosis is a rare disorder of the cutaneous dendritic cell group with a varied clinical presentation and evolution. The pathologic substrate is constituted by the proliferation of indeterminate cells (ICs) that are immunophenotypically characterized by the positivity of CD1a, CD68, and faint/focal S100, plus the negativity for CD207 (langerin). The authors present the case of a healthy elderly woman who presented generalized dome-shaped reddish cutaneous nodules over her trunk, neck, face, and extremities over a period of 18 months. A laboratory and imaging work-up ruled out internal involvement. The skin biopsy was consistent with IC histiocytosis. The patient was treated with narrowband ultraviolet B phototherapy, which resulted in an excellent short-term outcome. PMID:27547741

  18. Long-term sequelae of histiocytosis X.

    PubMed

    Komp, D M

    1981-01-01

    Residual disabilities are seen in more than half of children who survive histiocytosis. These problems are secondary to either continuously active disease or scarring of previously affected tissues. Fatal outcome from disabilities are particularly seen with pulmonary disease, either from progressive fibrosis or complicating opportunistic infections. "Second' tumors have been seen in association with radiation: brain tumors, osteosarcoma, and thyroid carcinoma. No cases of chemotherapy-related malignancy have been reported. PMID:6975581

  19. Langerhans Cell Histiocytosis of the Rib in an Adult: A Case Report

    PubMed Central

    Kim, Sung Hyun; Choi, Moon Young

    2016-01-01

    Single-site, single-system Langerhans cell histiocytosis (LCH) of the rib is one of the rarest causes of bone tumor in adults. Herein, we report a case of a healthy 35-year-old male who presented with upper back pain that was attributed to a solitary osteolytic lesion at the posterolateral aspect of his sixth rib. For diagnostic confirmation and treatment, partial resection of the sixth rib was performed and pathologic finding was consistent with LCH. At the final follow-up after 2 years, no local recurrence or metastasis was observed. PMID:26933424

  20. A Rare Case of Langerhans Cell Histiocytosis of the Skull in an Adult: a Systematic Review

    PubMed Central

    Chiong, Corinna; Jayachandra, Shruti; D. Eslick, Guy; Al-Khawaja, Darweesh; Casikar, Vidyasagar

    2013-01-01

    We report a 41-year old male who presented to the Emergency Department after falling while water-skiing. He had a previous medical history included chronic headaches, which had persisted for the last 2-3 months prior to presentation. Computed tomography of the head showed a small hypersensitivity with a small extra axial collection with a maximum thickness of 1mm. Differential diagnoses included an arachnoid cyst, haemangioma, meningioma or a secondary lesion. A diagnosis of Langerhans Cell Histiocytosis was made based on the histopathology examination and the immunoperoxidase staining. PMID:24179650

  1. The potential cost-effectiveness of the Diamondback 360® Coronary Orbital Atherectomy System for treating de novo, severely calcified coronary lesions: an economic modeling approach

    PubMed Central

    Chambers, Jeffrey; Généreux, Philippe; Lee, Arthur; Lewin, Jack; Young, Christopher; Crittendon, Janna; Mann, Marita; Garrison, Louis P.

    2015-01-01

    Background: Patients who undergo percutaneous coronary intervention (PCI) for severely calcified coronary lesions have long been known to have worse clinical and economic outcomes than patients with no or mildly calcified lesions. We sought to assess the likely cost-effectiveness of using the Diamondback 360® Orbital Atherectomy System (OAS) in the treatment of de novo, severely calcified lesions from a health-system perspective. Methods and results: In the absence of a head-to-head trial and long-term follow up, cost-effectiveness was based on a modeled synthesis of clinical and economic data. A cost-effectiveness model was used to project the likely economic impact. To estimate the net cost impact, the cost of using the OAS technology in elderly (⩾ 65 years) Medicare patients with de novo severely calcified lesions was compared with cost offsets. Elderly OAS patients from the ORBIT II trial (Evaluate the Safety and Efficacy of OAS in Treating Severely Calcified Coronary Lesions) [ClinicalTrials.gov identifier: NCT01092426] were indirectly compared with similar patients using observational data. For the index procedure, the comparison was with Medicare data, and for both revascularization and cardiac death in the following year, the comparison was with a pooled analysis of the Harmonizing Outcomes with Revascularization and Stents in Acute Myocardial Infarction (HORIZONS-AMI)/Acute Catheterization and Urgent Intervention Triage Strategy (ACUITY) trials. After adjusting for differences in age, gender, and comorbidities, the ORBIT II mean index procedure costs were 17% (p < 0.001) lower, approximately US$2700. Estimated mean revascularization costs were lower by US$1240 in the base case. These cost offsets in the first year, on average, fully cover the cost of the device with an additional 1.2% cost savings. Even in the low-value scenario, the use of the OAS is cost-effective with a cost per life-year gained of US$11,895. Conclusions: Based on economic modeling

  2. Solitary Langerhans cell histiocytosis of frontal lobe: a case report and literature review

    PubMed Central

    Cai, Shanshan; Zhang, Sheng; Liu, Xueyong; Lin, Yuanxiang; Wu, Chunlin; Chen, Yupeng; Hu, Jianping

    2014-01-01

    The brain parenchymal Langerhans cell histiocytosis (LCH) without systemic disease or lytic skull lesions is extremely rare. We report a 23-year-old male presenting with new onset 1 hour seizure with loss of consciousness 20 days prior to admission, and recurrent seizure 2 weeks later. Brain magnetic resonance imaging (MRI) showed an irregularly mass with enhancement involving the right frontal lobe. Microscopically, the lesion was characterized by sheets of Langerhans cells in addition to reactive inflammatory elements. Immunohistochemically, Langerhans cells were positive for Langerin, CD1a and S-100 protein. The patient received no chemotherapy or radiotherapy after surgery. After 24 months of follow-up, no recurrence or other systemic lesions were observed. Although there is no standard treatment for solitary cerebral LCH, the prognosis generally appears to be good. PMID:24826063

  3. Atypical histiocytosis in the lung

    PubMed Central

    Cao, Weijun; Zhang, Rongxuan; Zhou, Ying; Xu, Jinfu; Garfield, David H.

    2013-01-01

    Objective To report a rare case of atypical histiocytic tumor of the lung with a review of literature. Methods The clinical materials were noted. Literature related to this condition from the past 50 years was reviewed from the group of histiocytic tumors. Results and conclusions Clinical manifestations were non-specific. The imaging characteristics of our case were infiltrative lesions with multiple cysts in both lungs. Pathology showed nodular proliferation of atypical cells. Immunohistochemistry suggested a histiocytic origin of the infiltrating atypical cells. Because the pathological findings did not fall into any particular category of typical histiocytic tumors, the final diagnosis was atypical histiocytic tumor. The presentation of atypical histiocytic tumor of the lungs, only, with infiltrative lesions and multiple air cysts seems very rare, with pathological examination being “gold standard” for the diagnosis. PMID:23991320

  4. Pulmonary langerhans cell histiocytosis: case series and literature review.

    PubMed

    Wei, Ping; Lu, Hai-Wen; Jiang, Sen; Fan, Li-Chao; Li, Hui-Ping; Xu, Jin-Fu

    2014-11-01

    Pulmonary Langerhans cell histiocytosis (PLCH) is a rare disease with insidious onset and nonspecific manifestations. The objective of this article was to characterize the clinical manifestations and features of PLCH by retrospectively analyzing clinical data of patients with PLCH in addition to simultaneous review of literature.A retrospective analysis was conducted on clinical data of patients with PLCH (n = 7), whose conditions were diagnosed by biopsy from pulmonary tissue (n = 6) or enlarged lymph nodes in the neck (n = 1) and confirmed by PLCH typical radiological features on computed tomography (CT) scan, between January 2001 and September 2012 at the Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China. The review of published reports was made to further emphasize the clinical manifestation and radiological features of PLCH.Long history of cigarette smoking was found in 6 patients. Two patients had recurrent pneumothorax and the other 2 had pulmonary arterial hypertension (World Health Organization group 5 pulmonary hypertension), diagnosed through ultrasonic cardiogram. The nodular shadows were revealed by chest CT scan in 5 patients, cystic shadows in 5 patients, and reticular shadows in 2 patients, as major manifestations, respectively; most of the lesions were located in the middle or upper segments of the lung. The obvious shrank of lesion was found in 1 patient after completely quitting smoking.The pathogenesis of PLCH might be closely associated with smoking. The cystic or nodular lesion was the typical radiological features. Further prospective studies with large sample size are required to further validate the study results and understand the clinical characteristics of PLCH to avoid misdiagnosis. PMID:25415669

  5. Pulmonary Langerhans Cell Histiocytosis: Case Series and Literature Review

    PubMed Central

    Wei, Ping; Lu, Hai-Wen; Jiang, Sen; Fan, Li-Chao; Li, Hui-Ping; Xu, Jin-Fu

    2014-01-01

    Abstract Pulmonary Langerhans cell histiocytosis (PLCH) is a rare disease with insidious onset and nonspecific manifestations. The objective of this article was to characterize the clinical manifestations and features of PLCH by retrospectively analyzing clinical data of patients with PLCH in addition to simultaneous review of literature. A retrospective analysis was conducted on clinical data of patients with PLCH (n = 7), whose conditions were diagnosed by biopsy from pulmonary tissue (n = 6) or enlarged lymph nodes in the neck (n = 1) and confirmed by PLCH typical radiological features on computed tomography (CT) scan, between January 2001 and September 2012 at the Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China. The review of published reports was made to further emphasize the clinical manifestation and radiological features of PLCH. Long history of cigarette smoking was found in 6 patients. Two patients had recurrent pneumothorax and the other 2 had pulmonary arterial hypertension (World Health Organization group 5 pulmonary hypertension), diagnosed through ultrasonic cardiogram. The nodular shadows were revealed by chest CT scan in 5 patients, cystic shadows in 5 patients, and reticular shadows in 2 patients, as major manifestations, respectively; most of the lesions were located in the middle or upper segments of the lung. The obvious shrank of lesion was found in 1 patient after completely quitting smoking. The pathogenesis of PLCH might be closely associated with smoking. The cystic or nodular lesion was the typical radiological features. Further prospective studies with large sample size are required to further validate the study results and understand the clinical characteristics of PLCH to avoid misdiagnosis. PMID:25415669

  6. Liver involvement of Langerhans’ cell histiocytosis in children

    PubMed Central

    Yi, Xiaoping; Han, Tong; Zai, Hongyan; Long, Xueying; Wang, Xiaoyi; Li, Wenzheng

    2015-01-01

    Objective: Liver involvement is relatively frequent in children with Langerhans cell histiocytosis (LCH). Its features remain poorly defined. Methods: A retrospective study was carried out on 14 hepatic LCH children in our hospital. The Clinicopathological and radiological features of this disease was discussed. Results: The rate of liver involvement in children LCH patients is 51.9%. Majority of the patients were disseminated cases. Hepatomegaly was clinically confirmed in 11 cases (78.6%). Liver function dysfunction was seen in nine (64.3%) children. The association of multi-modal imaging significantly yielded more diagnostic information. There are some imaging characteristics of this disease, CT and MRI could help to assess the staging, extent of the hepatic lesions. We found that liver involvement had a significant impact on survival. Patients treated with systemic chemotherapy earlier from time of diagnosis had a relatively better outcome. Conclusions: The rate of liver involvement in children LCH patients maybe much higher than that of expected. We suggest that clinical and biological liver evaluation and abdominal imaging must be performed regularly onwards to screen every LCH children patient from the time of the initial diagnosis. Patient should be treated with systemic chemotherapy earlier. PMID:26221247

  7. Pulmonary Langerhans Cell Histiocytosis: An Update From the Pathologists' Perspective.

    PubMed

    Roden, Anja C; Yi, Eunhee S

    2016-03-01

    Context .- Pulmonary Langerhans cell histiocytosis (PLCH) is a rare histiocytic disorder that almost exclusively affects the lungs of smokers. PLCH is characterized by bronchiolocentric nodules and/or cysts in an upper and mid lung distribution with sparing of the costophrenic angles. The diagnosis can be challenging and often requires transbronchial biopsy or surgical lung biopsy. Pulmonary hypertension is a relatively common and sometimes severe complication of PLCH. The pathogenesis of PLCH is still debated. Recently, BRAF V600E mutation and BRAF expression have been identified in some patients with PLCH, suggesting that at least a subset of PLCH has a clonal proliferation. While smoking cessation is the first-line treatment of PLCH, some patients might require additional treatment and eventually transplant. Given that the lesional cells of PLCH express BRAF in some patients, MAPKinase pathway-targeted treatment might be useful for therapy-resistant patients. Objective . -To present the more recently recognized clinical and pathologic aspects of PLCH, including pulmonary hypertension in PLCH, pathogenesis, and treatment, as well as the basic diagnostic approach to PLCH. Data Sources .- Authors' own research, and search of literature database (PubMed) and UpToDate. Conclusions . -Despite the recent progress, more studies are needed to elucidate the biology of PLCH for identification of prognostic factors and appropriate treatment options, especially for therapy-refractory PLCH cases. PMID:26927717

  8. [Pulmonary Langerhans cell histiocytosis in adults].

    PubMed

    Feuillet, Séverine; Giroux-Leprieur, Bénédicte; Tazi, Abdellatif

    2010-01-01

    Pulmonary Langerhans-cell histiocytosis in adults is a rare condition of unknown etiology characterized by the accumulation of Langerhans cells organized in granulomas involving the distal bronchioles and destroying their walls. It occurs in young subjects who smoke, with frequency peaking between 20 and 40 years. High-resolution thoracic CT is essential for diagnosis; in typical forms it shows a combination of nodules, cavitary nodules, thick-walled cysts, and thin-walled cysts. Diagnostic certainty requires a surgical lung biopsy, by videothoracoscopy, but only if a specialist considers it indicated. It is difficult to predict the disease course for any given patient. A prospective multicenter cohort study currently underway should provide more information about the natural history of this disease. Management is empirical, for efficacy has not been proved for any treatment. Stopping smoking is especially important to prevent the added development of chronic obstructive pulmonary disease (COPD), cardiovascular complications, or the onset of bronchopulmonary cancer, the frequency of which appears elevated in these patients. Oral corticosteroids are used to treat disease progression, especially in the symptomatic mainly nodular forms, but their efficacy for respiratory function has not been shown. Vinblastine, the reference treatment for multisystem forms of Langerhans-cell histiocytosis, is not indicated for pulmonary involvement in adults. Better knowledge of the pathogenic mechanisms involved in this condition should eventually make it possible to develop innovative treatment strategies. The creation of the national reference center for Langerhans-cell histiocytosis has given new momentum to clinical and pathophysiologic research on this orphan disease. PMID:19959324

  9. Pulmonary Langerhans Cell Histiocytosis with Lytic Bone Involvement in an Adult Smoker: Regression following Smoking Cessation.

    PubMed

    Routy, B; Hoang, J; Gruber, J

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare myeloid neoplasm characterized by the proliferation and dissemination of histiocytes. These in turn may cause symptoms ranging from isolated, infiltrative lesions to severe multisystem disease. Pulmonary Langerhans cell histiocytosis (PLCH) presents as a localized polyclonal proliferation of Langerhans cells in the lungs causing bilateral cysts and fibrosis. In adults, this rare condition is considered a reactive process associated with cigarette smoking. Recently, clonal proliferation has been reported with the presence of BRAF V600E oncogenic mutation in a subset of PLCH patients. Spontaneous resolution was described; however, based on case series, smoking cessation remains the most effective way to achieve complete remission and prevent long term complications related to tobacco. Herein, we report the case of an adult woman with biopsy-proven PLCH presenting with thoracic (T8) vertebral bone destruction. Both the lung and the bone diseases regressed following smoking cessation, representing a rare case of synchronous disseminated PCLH with bone localization. This observation underscores the contribution of cigarette smoking as a systemic trigger of both pulmonary and extrapulmonary bone lesions. A review of similar cases in the literature is also presented. PMID:25789184

  10. Pulmonary Langerhans Cell Histiocytosis with Lytic Bone Involvement in an Adult Smoker: Regression following Smoking Cessation

    PubMed Central

    Routy, B.; Hoang, J.; Gruber, J.

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare myeloid neoplasm characterized by the proliferation and dissemination of histiocytes. These in turn may cause symptoms ranging from isolated, infiltrative lesions to severe multisystem disease. Pulmonary Langerhans cell histiocytosis (PLCH) presents as a localized polyclonal proliferation of Langerhans cells in the lungs causing bilateral cysts and fibrosis. In adults, this rare condition is considered a reactive process associated with cigarette smoking. Recently, clonal proliferation has been reported with the presence of BRAF V600E oncogenic mutation in a subset of PLCH patients. Spontaneous resolution was described; however, based on case series, smoking cessation remains the most effective way to achieve complete remission and prevent long term complications related to tobacco. Herein, we report the case of an adult woman with biopsy-proven PLCH presenting with thoracic (T8) vertebral bone destruction. Both the lung and the bone diseases regressed following smoking cessation, representing a rare case of synchronous disseminated PCLH with bone localization. This observation underscores the contribution of cigarette smoking as a systemic trigger of both pulmonary and extrapulmonary bone lesions. A review of similar cases in the literature is also presented. PMID:25789184

  11. Langerhans Cell Histiocytosis of the Temporal Bone.

    PubMed

    Ginat, Daniel Thomas; Johnson, Daniel N; Cipriani, Nicole A

    2016-06-01

    Langerhans cell histiocytosis involving the temporal bone region is uncommon and can resemble malignant neoplasms on imaging due to high cellularity. Although recognizing the presence of sharp margins with beveled-edges can be helpful, tissue sampling is often necessary for confirming the diagnosis. Cytology classically demonstrates kidney-bean shaped nuclei within the Langerhans cells and immunohistochemical staining is positive for S-100, peanut agglutinin (PNA), MHC class II, CD1a, and Langerin (CD 207). These features are exemplified in this sine qua non radiology-pathology correlation article. PMID:25903273

  12. Langerhans cell histiocytosis in the adult.

    PubMed

    Malpas, J S; Norton, A J

    1996-12-01

    A study of 47 well-documented patients with Langerhans cell histiocytosis (LCH) showed a slight female preponderance, with onset as late as the ninth decade. The skin was the commonest site of presentation, but pulmonary and bone involvement was frequent. Patients with single-site disease did best. The worst prognosis was seen in the elderly or those with organ dysfunction. A high incidence of associated malignant disease was seen, which could precede, be coincidental with, or occur after a diagnosis of LCH. PMID:8888814

  13. Selective aspiration or neurotoxic lesions of orbital frontal areas 11 and 13 spared monkeys’ performance on the object discrimination reversal task

    PubMed Central

    Kazama, Andy; Bachevalier, Jocelyne

    2009-01-01

    Damage to the orbital frontal cortex (OFC) has long been associated with reversal learning deficits in several species. In monkeys, this impairment follows lesions that include several OFC subfields. However, the different connectional patterns of OFC subfields together with neuroimaging data in humans have suggested that specific OFC areas play distinctive roles in processing information necessary to guide behavior (Kringelbach and Rolls, 2004; Barbas, 2007; Price, 2007). More specifically, areas 11 and 13 contribute to a sensory network, whereas medial areas 10, 14, and 25 are heavily connected to a visceromotor network. To examine the contribution of areas 11 and 13 to reversal learning, we tested monkeys with selective damage to these two OFC areas on two versions of the ODR task using either 1 or 5 discrimination problems. We compared their performance with that of sham-operated controls and of animals with neurotoxic amygdala lesions, which served as operated controls. Neither damage to areas 11 and 13 nor damage to the amygdala affected performance on the ODR tasks. The results indicate that areas 11 and 13 do not critically contribute to reversal learning and that adjacent damage to OFC subfields (10, 12, 14 and 25) could account for the ODR deficits found in earlier lesion studies. This sparing of reversal learning will be discussed in relation to deficits found in the same animals on tasks that measure behavioral modulation when relative value of affective (positive and negative) stimuli was manipulated. PMID:19261875

  14. A rare case of solitary brain Langerhans cell histiocytosis with intratumoral hemorrhage in a patient affected by Turner syndrome

    PubMed Central

    Granata, Francesca; Morabito, Rosa; Grasso, Giovanni; Alafaci, Elisabetta; Salpietro, Francesco M.; Alafaci, Concetta

    2016-01-01

    Background: Langerhans cell histiocytosis (LCH) is a rare disease involving clonal proliferation of cells with characteristics similar to bone marrow-derived Langerhans cells. The case of a young woman, affected by Turner syndrome and a solitary intraparenchymal LCH associated with an osteolytic lesion of the overlying skull, is presented. Case Description: The patient, with an insidious history of headache and a growing soft mass in the left frontal region, presented with a sudden generalized tonic-clonic epileptic seizure. Neuroradiological investigations showed an osteolytic lesion of the left frontal bone and an underlying brain lesion associated with recent signs of bleeding. The patient was operated on with a complete removal of the lesion. The postoperative course was uneventful. Conclusions: The clinical, neuroradiological, and intraoperative findings are presented, along with a review of the literature. Although rare, LCH should be considered in the differential diagnosis when a scalp lesion occurs with a progressive growing. PMID:27127696

  15. Histiocytic sarcoma that mimics benign histiocytosis.

    PubMed

    Boisseau-Garsaud, A M; Vergier, B; Beylot-Barry, M; Nastasel-Menini, F; Dubus, P; de Mascarel, A; Eghbali, H; Beylot, C

    1996-06-01

    A 28-year-old man presented with a histiocytic sarcoma of a very uncommon origin, as it had developed for several years like a benign cutaneous histiocytosis resembling generalized eruptive histiocytoma before becoming acute, with nodal and massive pulmonary involvement. Despite various chemotherapies, the patient died within 8 months. Skin biopsies showed histiocytic proliferation in the dermis and node biopsies showed histiocytic proliferation with a sinusoidal pattern. Immunohistochemical analysis, performed on paraffin-embedded sections, demonstrated strong labeling of tumoral cells for CD68 and moderate labeling for CD3 and CD4. CD30 labeling was negative. S-100 protein was positive on a Langerhans' cell reactive subpopulation. Electron microscopy confirmed the histiocytic nature of malignant cells and showed cytoplasmic inclusions such as regularly laminated bodies, dense bodies and pleomorphic inclusions. No Birbeck granules were seen. A gene rearrangement study of T-cell receptor gamma and immunoglobulin heavy chain genes showed a germline configuration. Histiocytic sarcoma is an extremely rare true histiocytic malignancy, the existence of which has been recently debated since it has often been mistaken in the past for large cell lymphomas. Such a deceptive onset as benign cutaneous histiocytosis has not been described in the literature to our knowledge. PMID:8793665

  16. Case of Langerhans Cell Histiocytosis That Mimics Meningioma in CT and MRI

    PubMed Central

    Zhu, Ming; Yu, Bing-Bing; Zhai, Ji-Liang

    2016-01-01

    Langerhans cell histiocytosis (LCH) is a rare disorder histologically characterized by the proliferation of Langerhans cells. Here we present the case of a 13-year-old girl with LCH wherein CT and MRI results led us to an initially incorrect diagnosis of meningioma. The diagnosis was corrected to LCH based on pathology findings. An intracranial mass was found mainly in the dura mater, with thickening of the surrounding dura. It appeared to be growing downward from the calvaria, pressing on underlying brain tissue, and had infiltrated the inner skull, causing a bone defect. The lesion was calcified with the typical dural tail sign. The dural origin of the lesion was verified upon surgical dissection. There are no previous reports in the literature describing LCH of dural origin presenting in young patients with typical dural tail signs and meningioma-like imaging findings. The current case report underscores the need for thorough histological and immunocytochemical examinations in LCH differential diagnosis. PMID:26962425

  17. Case of Langerhans Cell Histiocytosis That Mimics Meningioma in CT and MRI.

    PubMed

    Zhu, Ming; Yu, Bing-Bing; Zhai, Ji-Liang; Sun, Gang

    2016-03-01

    Langerhans cell histiocytosis (LCH) is a rare disorder histologically characterized by the proliferation of Langerhans cells. Here we present the case of a 13-year-old girl with LCH wherein CT and MRI results led us to an initially incorrect diagnosis of meningioma. The diagnosis was corrected to LCH based on pathology findings. An intracranial mass was found mainly in the dura mater, with thickening of the surrounding dura. It appeared to be growing downward from the calvaria, pressing on underlying brain tissue, and had infiltrated the inner skull, causing a bone defect. The lesion was calcified with the typical dural tail sign. The dural origin of the lesion was verified upon surgical dissection. There are no previous reports in the literature describing LCH of dural origin presenting in young patients with typical dural tail signs and meningioma-like imaging findings. The current case report underscores the need for thorough histological and immunocytochemical examinations in LCH differential diagnosis. PMID:26962425

  18. Concomitant nodal involvement by Langerhans cell histiocytosis and Hodgkin's lymphoma.

    PubMed

    Geurten, Claire; Thiry, Albert; Jamblin, Paul; Demarche, Martine; Hoyoux, Claire

    2015-12-01

    A 10-year-old girl with a family history of Hodgkin's lymphoma presented with a 2 month history of cervical lymphadenopathy and weight loss. Biopsy indicated concomitant nodal involvement by Langerhans cell histiocytosis and Hodgkin's lymphoma. Such an association is rare, especially so in children, but is not an isolated phenomenon, thereby prompting the question of whether Langerhans cell histiocytosis is a reactive or a neoplastic process. PMID:26556799

  19. Pulmonary Langerhans Cell Histiocytosis in an Adult Male Presenting with Central Diabetes Insipidus and Diabetes Mellitus: A Case Report

    PubMed Central

    Choi, Yeun Seoung; Lim, Jung Soo; Kwon, Woocheol; Jung, Soon-Hee; Park, Il Hwan; Lee, Myoung Kyu; Lee, Won Yeon; Yong, Suk Joong; Lee, Seok Jeong; Jung, Ye-Ryung; Choi, Jiwon; Choi, Ji Sun; Jeong, Joon Taek; Yoo, Jin Sae

    2015-01-01

    Pulmonary Langerhans cell histiocytosis is an uncommon diffuse cystic lung disease in adults. In rare cases, it can involve extrapulmonary organs and lead to endocrine abnormalities such as central diabetes insipidus. A 42-year-old man presented with polyphagia and polydipsia, as well as a dry cough and dyspnea on exertion. Magnetic resonance imaging of the hypothalamic-pituitary system failed to show the posterior pituitary, which is a typical finding in patients with central diabetes insipidus. This condition was confirmed by a water deprivation test, and the patient was also found to have type 2 diabetes mellitus. Computed tomographic scanning of the lungs revealed multiple, irregularly shaped cystic lesions and small nodules bilaterally, with sparing of the costophrenic angles. Lung biopsy through video-assisted thoracoscopic surgery revealed pulmonary Langerhans cell histiocytosis. On a follow-up visit, only 1 year after the patient had quit smoking, clinical and radiological improvement was significant. Here, we report an uncommon case of pulmonary Langerhans cell histiocytosis that simultaneously presented with diabetes insipidus and diabetes mellitus. PMID:26508947

  20. Pulmonary Langerhans Cell Histiocytosis in an Adult Male Presenting with Central Diabetes Insipidus and Diabetes Mellitus: A Case Report.

    PubMed

    Choi, Yeun Seoung; Lim, Jung Soo; Kwon, Woocheol; Jung, Soon-Hee; Park, Il Hwan; Lee, Myoung Kyu; Lee, Won Yeon; Yong, Suk Joong; Lee, Seok Jeong; Jung, Ye-Ryung; Choi, Jiwon; Choi, Ji Sun; Jeong, Joon Taek; Yoo, Jin Sae; Kim, Sang-Ha

    2015-10-01

    Pulmonary Langerhans cell histiocytosis is an uncommon diffuse cystic lung disease in adults. In rare cases, it can involve extrapulmonary organs and lead to endocrine abnormalities such as central diabetes insipidus. A 42-year-old man presented with polyphagia and polydipsia, as well as a dry cough and dyspnea on exertion. Magnetic resonance imaging of the hypothalamic-pituitary system failed to show the posterior pituitary, which is a typical finding in patients with central diabetes insipidus. This condition was confirmed by a water deprivation test, and the patient was also found to have type 2 diabetes mellitus. Computed tomographic scanning of the lungs revealed multiple, irregularly shaped cystic lesions and small nodules bilaterally, with sparing of the costophrenic angles. Lung biopsy through video-assisted thoracoscopic surgery revealed pulmonary Langerhans cell histiocytosis. On a follow-up visit, only 1 year after the patient had quit smoking, clinical and radiological improvement was significant. Here, we report an uncommon case of pulmonary Langerhans cell histiocytosis that simultaneously presented with diabetes insipidus and diabetes mellitus. PMID:26508947

  1. Langerhans cell histiocytosis or tuberculosis on a medieval child (Oppidum de la Granède, Millau, France - 10th-11th centuries AD).

    PubMed

    Colombo, Antony; Saint-Pierre, Christophe; Naji, Stephan; Panuel, Michel; Coqueugniot, Hélène; Dutour, Olivier

    2015-06-01

    In 2008, a skeleton of a 1 - 2.5-year-old child radiocarbon dated from the 10th - 11th century AD was discovered on the oppidum of La Granède (Millau, France). It presents multiple cranial osteolytic lesions having punched-out or geographical map-like aspects associated with sequestrum and costal osteitis. A multi 3D digital approach (CT, μCT and virtual reconstruction) enabled us to refine the description and identify the diploic origin of the lytic process. Furthermore, precise observation of the extent of the lesions and associated reorganization of the skeletal micro-structure were possible. From these convergent pieces of evidence, the differential diagnosis led to three possibilities: Langerhans cell histiocytosis, tuberculosis, or Langerhans cell histiocytosis and tuberculosis. PMID:25747815

  2. Langerhans Cell Histiocytosis in an Adult with Involvement of the Calvarium, Cerebral Cortex and Brainstem: Discussion of Pathophysiology and Rationale for the Use of Intravenous Immune Globulin

    PubMed Central

    Dardis, Christopher; Aung, Thandar; Shapiro, William; Fortune, John; Coons, Stephen

    2015-01-01

    We report a case of Langerhans cell histiocytosis in a 64-year-old male who presented with symptoms and signs of brain involvement, including seizures and hypopituitarism. The diagnosis was confirmed with a biopsy of a lytic skull lesion. The disease affecting the bone showed no sign of progression following a short course of cladribine. Signs of temporal lobe involvement led to an additional biopsy, which showed signs of nonspecific neurodegeneration and which triggered status epilepticus. Lesions noted in the brainstem were typical for the paraneoplastic inflammation reported in this condition. These lesions improved after treatment with cladribine. They remained stable while on treatment with intravenous immune globulin. PMID:25873887

  3. Medication Management of Jaw Lesions for Dental Patients.

    PubMed

    Ogle, Orrett E; Santosh, Arvind Babu Rajendra

    2016-04-01

    Most pathologic lesions of the jaws or of oral mucosa are treated successfully by surgical interventions. For treatment of the central giant cell lesion, aneurysmal bone cysts, histiocytosis of the mandible, hemangioma, odontogenic keratocyst, Paget disease, oral submucous fibrosis, and oral lichen planus, medical management consisting of intralesional injections, sclerosing agents, and systemic bisphosphonates is as successful as surgical procedures with fewer complications. Pharmacology of agents used and protocols are presented. PMID:27040297

  4. [Langerhans cell histiocytosis causing cervical myelopathy].

    PubMed

    Doléagbénou, A K; Mukengeshay Ntalaja, J; Derraz, S; El Ouahabi, A; El Khamlichi, A

    2012-08-01

    Langerhans cell histiocytosis (LCH), a disorder of the phagocytic system, is a rare condition. Moreover, spinal involvement causing myelopathy is even rare and unusual. Here, we report a case of atypical LCH causing myelopathy, which was subsequently treated by corporectomy and fusion. An 8-year-old boy presented with 3 weeks of severe neck pain and limited neck movement accompanying upper and lower limbs motor weakness. CT scans revealed destruction of C5 body and magnetic resonance imaging showed a tumoral process at C5 with cord compression. Interbody fusion using anterior cervical plate packed by autologus iliac bone was performed. Pathological examination confirmed the diagnosis of LCH. After the surgery, the boy recovered from radiating pain and motor weakness of limbs. Despite the rarity of the LCH in the cervical spine, it is necessary to maintain our awareness of this condition. When neurologic deficits are present, operative treatment should be considered. PMID:22552159

  5. Cytomegalovirus and Langerhans Cell Histiocytosis: Is There a Link?

    PubMed Central

    Khoddami, Maliheh; Nadji, Seyed-Alireza; Dehghanian, Paria; Vahdatinia, Mahsa; Shamshiri, Ahmad-Reza

    2016-01-01

    Background: Langerhans cell histiocytosis is a rare proliferative histiocytic disease of unknown etiology. Histologically, it is characterized by granuloma-like proliferation of Langerhans-type dendritic cells derived from bone marrow. Many investigators have suggested the possible role of viruses such as Epstein-Barr virus, human herpesvirus-6 (HHV-6), herpes simplex virus (HSV) types 1 and 2, and Cytomegalovirus in the pathogenesis of Langerhans cell histiocytosis. Objectives: In this study, we have investigated the presence of Cytomegalovirus in Langerhans cell histiocytosis in Iranian children. Patients and Methods: In this retrospective study, we have investigated the presence of Cytomegalovirus DNA expression, using paraffin-embedded tissue samples of 30 patients with Langerhans cell histiocytosis and 30 age and site-matched controls by qualitative Polymerase Chain Reaction (PCR) method. Results: No significant difference in prevalence of Cytomegalovirus presence between patients and controls was found. Cytomegalovirus was found by qualitative PCR in only 2 (6.66%) out of 30 patients and in 1 (3.3%) of 30 control samples with a P value of 1 (1.00 > 0.05) using chi-square test with OR: 2.07; 95% CI of OR: 0.18 - 24.15. Conclusions: Our findings do not support the hypothesis of a possible role for Cytomegalovirus in the pathogenesis of Langerhans cell histiocytosis. PMID:27307972

  6. Pulmonary Langerhans Cell Histiocytosis in an Adult Diagnosed with Solitary Inguinal Lymphadenopathy.

    PubMed

    Luo, Wei; Li, Ya-lun; Jia, Lian; Liu, Chun-tao

    2015-01-01

    We herein report an extremely rare case of pulmonary Langerhans cell histiocytosis with a solitary enlarged inguinal lymph node. A 19-year-old man presented with a non-productive cough lasting for over a five-month period and an enlarged left inguinal lymph node that had persisted for four months. A histopathological study of the lymph node specimens found Langerhans cells coupled with eosinophils. Positive immunohistochemical staining for langerin, Cluster of Differentiation 1a, S100 in the Langerhans cells confirmed the diagnosis, and a mildly impaired ventilation function in addition to multiple peripheral pulmonary cystic lesions were detected. The patient was managed with prednisone (0.5 mg/kg daily), with slow tapering over several months. PMID:26134198

  7. Sudden death of a patient with pulmonary Langerhans cell histiocytosis.

    PubMed

    Nakhla, Hassan; Jumbelic, Mary I

    2005-06-01

    We report a case of sudden death due to bilateral pneumothorax in a previously healthy 16-year-old adolescent white girl. She presented with sudden onset of shortness of breath followed by loss of consciousness. Postmortem chest radiograph showed bilateral pneumothoraces. Autopsy confirmed the bilateral pneumothorax and additionally showed emphysematous changes and bullae throughout the lung tissue. Microscopic sections of the lungs showed Langerhans cell histiocytosis. To the best of our knowledge, this is the first reported case of fatal presentation of pulmonary Langerhans cell histiocytosis. PMID:15913433

  8. Malignant histiocytosis. A phenotypic and genotypic investigation.

    PubMed Central

    Cattoretti, G.; Villa, A.; Vezzoni, P.; Giardini, R.; Lombardi, L.; Rilke, F.

    1990-01-01

    Ten cases of malignant histiocytosis (MH) were evaluated for clinical and histopathologic features, phenotype, and rearrangement of T cell receptor (TCR) beta, gamma, and alpha and immunoglobulin (Ig) genes (7/10). All cases were HLA-DR+ and CD30-positive. Four cases had molecular evidence of T cell lineage such as TCR beta, gamma, and alpha rearrangements, and one additional case synthesized the cytoplasmic TCR beta chain. The remaining five cases did not show unequivocal T, B, natural killer (NK) cell, or macrophagic origin, and three of them had germline TCR and Ig genes. Ultrastructural analysis was not helpful for the definition of the cell lineage. Most myelomonocytic markers (MAC387, CD13, CD14, CD64, CD68) were either negative on the MH cells or were expressed on cells with rearranged TCR gene. Precursor (CD34, CD7) and NK (CD16, CD56, and CD57) cell markers were not found. The lineage of a number of cases of MH remains unresolved. Images Figure 1 Figure 2 Figure 3 Figure 4 Figure 5 Figure 6 Figure 7 Figure 8 Figure 9 Figure 10 PMID:2349962

  9. Langerhans Cell Histiocytosis Presenting as Uncontrolled Asthma

    PubMed Central

    Rawlins, Frederic A.; Hull, James E.; Morgan, Julia A.; Morris, Michael J.

    2013-01-01

    Langerhans cell histiocytosis (LCH) is an uncommon disorder affecting primarily young adult smokers. It is characterized by abnormal proliferation of Langerhans cells, specialized monocyte-macrophage lineage antigen-presenting cells. LCH can affect the lungs in isolation or as part of a systemic disease. Most commonly, the disease presents in the third or fourth decade without gender predominance. Symptoms typically include dyspnea and cough. Commonly, physical examination is unremarkable but cor pulmonale may be observed in advanced disease. The chest radiograph is typically abnormal with nodular or interstitial infiltrates and cystic changes. High-resolution computed tomography of the chest with these findings in the middle and upper lobes of an adult smoker is virtually diagnostic of LCH. Pulmonary function assessment is variable. Asthma has rarely been reported in association with this disorder. There are only three reported cases of the diagnosis of concomitant asthma which have been made in association with the diagnosis of LCH. We present a case in which our patient presented with signs and symptoms of asthma to include confirmatory findings of airway hyperresponsiveness. The diagnosis of LCH was established after the patient failed to respond to conventional treatment for asthma, and further evaluation was completed. PMID:24065995

  10. Tertiary lymphoid structures are confined to patients presenting with unifocal Langerhans Cell Histiocytosis.

    PubMed

    Quispel, Willemijn T; Steenwijk, Eline C; van Unen, Vincent; Santos, Susy J; Koens, Lianne; Mebius, Reina; Egeler, R Maarten; van Halteren, Astrid G S

    2016-08-01

    Langerhans cell histiocytosis (LCH) is a neoplastic myeloid disorder with a thus far poorly understood immune component. Tertiary lymphoid structures (TLS) are lymph node-like entities which create an immune-promoting microenvironment at tumor sites. We analyzed the presence and clinical relevance of TLS in n = 104 H&E-stained, therapy-naive LCH lesions of non-lymphoid origin and applied immunohistochemistry to a smaller series. Lymphoid-follicular aggregates were detected in 34/104 (33%) lesions. In line with the lymphocyte recruitment capacity of MECA-79(+) high endothelial venules (HEVs), MECA-79(+)-expressing-LCH lesions (37/77, 48%) contained the most CD3(+) T-lymphocytes (p = 0.003). TLS were identified in 8/15 lesions and contained T-and B-lymphocytes, Follicular Dendritic Cells (FDC), HEVs and the chemokines CXCL13 and CCL21 representing key cellular components and TLS-inducing factors in conventional lymph nodes (LN). Lymphoid-follicular aggregates were most frequently detected in patients presenting with unifocal LCH (24/70, 34%) as compared to patients with poly-ostotic or multi-system LCH (7/30, 23%, p = 0.03). In addition, patients with lymphoid-follicular aggregates-containing lesions had the lowest risk to develop new LCH lesions (p = 0.04). The identification of various stages of TLS formation within LCH lesions may indicate a key role for the immune system in controlling aberrant histiocytes which arise in peripheral tissues. PMID:27622056

  11. Localized Langerhans cell histiocytosis masquerading as Brodie's abscess in a 2-year-old child: a case report

    PubMed Central

    Chang, Wei-Fang; Hsu, Yi-Chih; Wu, Yi-Der; Kuo, Chun-Lang; Huang, Guo-Shu

    2016-01-01

    Langerhans cell histiocytosis (LCH), formerly known as histiocytosis X, refers to a spectrum of diseases characterized by idiopathic proliferation of histiocytes that produce either focal (localized LCH) or systemic manifestations (Hand-Schüller-Christian disease and Letterer-Siwe disease). Localized LCH accounts for approximately 60-70 % of all LCH cases. Osseous involvement is the most common manifestation and typically involves the flat bones, along with lesions of the skull, pelvis, and ribs. Localized LCH in bone shows a wide spectrum of clinical manifestations and radiologic features that may mimic those of infections as well as benign and malignant tumors. The diagnostic imaging findings of localized LCH are also diverse and challenging. The penumbra sign is a common and characteristic magnetic resonance imaging (MRI) feature of Brodie's abscess, but is rarely seen in localized LCH. In this report, we describe a case of localized LCH misdiagnosed as Brodie's abscess in a 2-year-old child based on clinical symptoms, laboratory findings, and pre-diagnostic MRI findings (penumbra sign). Therefore, the penumbra sign is not sufficient to clearly establish the diagnosis of Brodie's abscess, and the differential diagnosis of localized LCH should be considered when a child with an osteolytic lesion presents with a penumbra sign. PMID:27065773

  12. Unusual lesions of the mediastinum

    PubMed Central

    Shamsuddin, Fatima; Khadilkar, Urmila N; Saha, Debarshi

    2015-01-01

    Objectives: To study unusual lesions in the mediastinum, which do not originate from the thymus, lymph nodes, neural tissues or germ cells, and tissues that normally engender pathologic lesions in the mediastinum. Materials and Methods: Of the 65 cases seen, 12 unusual lesion were encountered in a 5½ year period from 2006 to 2011. Results: Two cases of nodular colloid goiter and one each of the mediastinal cyst, undifferentiated carcinoma, and Langerhans cell histiocytosis (LCH) affected the anterosuperior mediastinum. In the middle mediastinum, one case each of the mesothelioma, malignant gastrointestinal stromal tumor (GIST), squamous cell carcinoma (SCC), solitary fibrous tumor (SFT), and pleomorphic sarcoma (PS) was seen. One case of meningeal melanocytoma (Mme) and primary pleural liposarcoma (PL) involved the posterior mediastinum. Persistent disease was seen in LCH after 2 years. Of all the cases with malignant lesions, only the patient with SCC was alive after 1 year. Conclusion: The cases of primary and SCC, LCH, melanocytoma, liposarcoma and PS, and GIST are unexpected and very rarely have paradigms in the mediastinum. Radiologic impression and knowledge of the compartment where these lesions arose from hardly assisted in arriving at a definitive opinion as the lesions were not typical of this location. A high index of suspicion and the immunohistochemical profile facilitated the final diagnosis. PMID:26664161

  13. Radiotherapy for Langerhans Cell Histiocytosis of Bilateral Eyelids

    PubMed Central

    Bourque, Jean-Marc; Lukovic, Jelena; Dar, A. Rashid

    2016-01-01

    Langerhans cell histiocytosis (LCH) is a rare disorder with numerous clinicopathological variants with differing clinical courses, treatment methods, and prognoses. We report one patient with atypical LCH of the bilateral lower eyelids and subsequent successful treatment with local radiation therapy. PMID:27004151

  14. Radiotherapy for Langerhans Cell Histiocytosis of Bilateral Eyelids.

    PubMed

    Leveson, James; Bourque, Jean-Marc; Lukovic, Jelena; Dar, A Rashid

    2016-01-01

    Langerhans cell histiocytosis (LCH) is a rare disorder with numerous clinicopathological variants with differing clinical courses, treatment methods, and prognoses. We report one patient with atypical LCH of the bilateral lower eyelids and subsequent successful treatment with local radiation therapy. PMID:27004151

  15. Radiographic and Pathologic Manifestations of Uncommon and Rare Pulmonary Lesions.

    PubMed

    Pfeifer, Kyle; Mian, Ali; Adebowale, Adeniran; Alomari, Ahmed; Kalra, Vivek; Krejci, Elise; Shin, Myung Soo

    2016-05-01

    Pulmonary opacities/nodules are common findings on computed tomography examinations, which may represent an underlying infections or malignancy. However, not every pulmonary nodule or opacity represents malignancy or infection. We present a pictorial essay illustrating common as well as obscure noninfectious, nonmalignant pulmonary lesions. Lesions discussed include organizing pneumonia, Langerhans cell histiocytosis, pulmonary amyloidosis, hyalinizing granuloma, tumourlet (benign localized neuroendocrine cell proliferations), atypical alveolar hyperplasia, inflammatory myofibroblastic tumour, papillary alveolar adenoma, plasma cell granuloma, juvenile xanthogranuloma, and sclerosing hemangiomas. We discuss the clinical presentation, prevalence, radiographic clues, pathology, and diagnostic pitfalls of these rare lesions. PMID:26690551

  16. Swelling of bilateral parotid glands: An unusual symptom of multisystem Langerhans cell histiocytosis

    PubMed Central

    Tang, Mengjie; Sheng, Qi; Zhu, Xiaodong; Zhang, Qin

    2014-01-01

    Objective: Langerhans cell histiocytosis is an unusual disorder of unknown etiology with heterogeneous clinical behaviors and variable outcomes. It can involve one or more organs or systems, but to our best knowledge, parotid glands involvement in Langerhans cell histiocytosis is extremely rare in English literature. Method: We report a 13-month-old girl who presented with bilateral parotid swelling as presenting symptom. She was misdiagnosed for 4 months, but final diagnosis was multisystem Langerhans cell histiocytosis. Result: After being treated for 18 months, the patient acquired complete remission and attained similar growth status to other healthy children. Conclusion: Langerhans cell histiocytosis may involve any organ; in patients with parotid enlargement, Langerhans cell histiocytosis should be kept in mind in the differential diagnoses. We emphasize the importance of biopsy for histologic evaluation as soon as possible and even repeatedly if initial results are negative for Langerhans cell histiocytosis. PMID:27489645

  17. Strategies for the Prevention of Central Nervous System Complications in Patients with Langerhans Cell Histiocytosis: The Problem of Neurodegenerative Syndrome.

    PubMed

    Imashuku, Shinsaku; Arceci, Robert J

    2015-10-01

    Diseases of the central nervous system (CNS) are common in patients with Langerhans cell histiocytosis (LCH). Besides active LCH lesions, neurodegenerative (ND) lesions of the cerebellum and/or basal ganglia may occur as late sequelae of LCH. While the etiology of this ND disease remains unclear, biomarkers in cerebrospinal fluid (CSF) may reflect the activity of CNS disease in these patients. However, no well-planned CSF studies have yet been performed in patients at high risk for ND-CNS-LCH. Potential parallels with other neuroinflammatory/neurodegenerative disease suggest the utility of examining these other disorders in establishing strategies for the prevention and/or treatment of ND-CNS-LCH. PMID:26461148

  18. Langerhans’ cell histiocytosis involving posterior elements of the dorsal spine: An unusual cause of extradural spinal mass in an adult

    PubMed Central

    Tyagi, Devendra K.; Balasubramaniam, Srikant; Savant, Hemant V.

    2011-01-01

    Langerhans cell histiocytosis (LCH) is a clonal proliferation of Langerhans cells occurring as an isolated lesion or as part of a systemic proliferation. It is commoner in children younger than 10 years of age with sparing of the posterior elements in more than 95% of cases. We describe a case of LCH in an adult female presenting with paraplegia. MRI revealed a well-defined extradural contrast enhancing mass at D2-D4 vertebral level involving the posterior elements of spine. D2-5 laminectomy with excision of lesion was performed which lead to marked improvement of patients neurological status. Histopathology was suggestive of eosinophilic granuloma. We describe the case, discuss its uniqueness and review the literature on this rare tumor presentation. PMID:23125497

  19. Adult Onset of BRAFV600E-Mutated Langerhans Cell Histiocytosis with Cutaneous Involvement Successfully Diagnosed by Immunohistochemical Staining

    PubMed Central

    Tono, Hisayuki; Fujimura, Taku; Kakizaki, Aya; Furudate, Sadanori; Ishibashi, Masaya; Aiba, Setsuya

    2015-01-01

    Langerhans cell histiocytosis (LCH) is characterized by the clonal proliferation of Langerhans cells; it is categorized as a single-system disease with single or multifocal lesions, and as a multi-system disease with or without the risk of organ involvement. Although the skin is not categorized as a risk organ, the precise diagnosis of skin lesions is necessary to determine the protocol for the treatment of LCH. In this report, we describe a 28-year-old Japanese man with adult onset of BRAFV600E-mutated LCH with cutaneous involvement successfully diagnosed by immunohistochemical staining. Our report suggests that immunohistochemical staining for the BRAFV600E gene could be a diagnostic tool to determine the clinical type of LCH. PMID:26500535

  20. Canine cutaneous histiocytoma is an epidermotropic Langerhans cell histiocytosis that expresses CD1 and specific beta 2-integrin molecules.

    PubMed Central

    Moore, P. F.; Schrenzel, M. D.; Affolter, V. K.; Olivry, T.; Naydan, D.

    1996-01-01

    Canine cutaneous histiocytoma (CCH) is a common, benign neoplasm of the dog. Histiocytomas most commonly occur as solitary lesions that undergo spontaneous regression. The age-specific incidence rate for histiocytomas drops precipitously after 3 years, although histiocytomas occur in dogs of all ages. Langerhans cells (LCs) in humans and dogs express abundant major histocompatibility complex class II molecules and a variety of leukocyte antigens characteristic of dendritic cell differentiation including CD1a, CD1b, CD1c, and CD11c. The immunophenotype of CCH resembled that of cutaneous LCs by virtue of the expression of CD1 molecules (CD1a, -b, and -c), CD11c, and major histocompatibility complex class II. Furthermore, histiocytoma cells had a tropism for epidermis, which was also consistent with an epidermal LC lineage. The expression of adhesion molecules such as CD11b (variable), CD44, CD54 (ICAM-1), and CD49d (VLA-4) in CCH indicated that the infiltrating cells had some of the characteristics of activated LCs, as these molecules are not expressed by normal, resting canine epidermal LCs. CCH did not express Thy-1 or CD4. Thy-1 expression is a characteristic of human and canine dermal dendrocytes, which are perivascular dendritic antigen-presenting cells closely related to epidermal LCs. CD4 expression is prevalent in human LC histiocytosis, and in this respect CCH differed from human LC histiocytosis. Here we demonstrate that CCH is a localized form of self-limiting LC histiocytosis, which predominantly expresses an epidermal LC phenotype. CCH occurs as solitary or, less commonly, as multiple cutaneous nodules or plaques, which rarely may extend beyond the skin to local lymph nodes. Regression of CCH occurs spontaneously in the vast majority of cases in primary and secondary sites, and is mediated by CD8+ alpha beta T cells. The high frequency of CCH within the general canine population offers the potential that the dog may provide an interesting model system to

  1. Role of multidisciplinary approach in a case of Langerhans cell histiocytosis with initial periodontal manifestations

    PubMed Central

    Cisternino, Angelo; Asa’ad, Farah; Fusco, Nicola; Ferrero, Stefano; Rasperini, Giulio

    2015-01-01

    Introduction: Langerhans cell histiocytosis (LCH) is a rare inflammatory myeloid neoplasia of unknown etiology occurring in both children and adults. This condition is characterized by an abnormal proliferation of Langerhans cells that may virtually affect all sites in the human body. Oral manifestations of LCH could be the first clinical sign of disease and its periodontal localization could be easily mistaken for other more common entities, such as chronic periodontitis, aggressive periodontitis, and necrotizing ulcerative periodontitis. Case presentation: A 32-years old female visited a private dental practice with a chief complaint of sensitivity in the mandibular left first molar. Clinical and radiographic examination revealed deep periodontal pocket, recession, furcation involvement, mobility, severe alveolar bone destruction and a diagnosis of aggressive periodontitis was rendered. Multiple tooth extractions were carried out due to progressive periodontal destruction with impaired healing and development of ulcerative lesions. Multidisciplinary investigation demonstrated that the periodontal involvement was a manifestation of an underlying systemic disease. A biopsy of a bone lesion was therefore performed, revealing the presence of multifocal single system LCH. Conclusion: The identification of periodontal LCH is not trivial given that it may clinically resemble other periodontal disease entities. The dentist can be the first health care personnel to unravel the presence of an underlying systemic LCH. PMID:26722570

  2. A Case of Langerhans Cell Histiocytosis Manifested as a Suprasellar Mass

    PubMed Central

    Yoon, Ju Young; Park, Byung-Kiu; Yoo, Heon; Lee, Sang Hyun; Hong, Eun Kyung; Park, Weon Seo; Kwon, Young Joo; Yoon, Jong Hyung

    2016-01-01

    Langerhans cell histiocytosis (LCH) has diverse clinical manifestations, including intracranial mass lesions. We report a case of LCH that manifested as a suprasellar mass, and initially misdiagnosed as a germ cell tumor. A 29-year-old woman presented with polyuria, polydipsia and amenorrhea. Laboratory findings revealed hypopituitarism with central diabetes insipidus, and a suprasellar mass and a pineal mass were observed on magnetic resonance imaging. Under the clinical impression of a germ cell tumor, the patient was treated with germ cell tumor chemotherapy (cisplatin and etoposide) and radiation therapy without biopsy. After initial shrinkage of the lesions, further growth of the tumor was observed and a biopsy was performed. The histopathology revealed LCH. After chemotherapy according to the LCH III protocol, the tumor disappeared. She is on regular follow up for 5 years without relapse. The present findings indicate that LCH should be included in the differential diagnosis of a suprasellar mass, even in adults, especially when it manifests with diabetes insipidus. This case also underscores the importance of a histopathologic diagnosis in patients with suprasellar tumors before the initiation of a specific therapy, even if the clinical findings are highly suggestive of a specific diagnosis. PMID:27195259

  3. A Case of Langerhans Cell Histiocytosis Manifested as a Suprasellar Mass.

    PubMed

    Yoon, Ju Young; Park, Byung-Kiu; Yoo, Heon; Lee, Sang Hyun; Hong, Eun Kyung; Park, Weon Seo; Kwon, Young Joo; Yoon, Jong Hyung; Park, Hyeon Jin

    2016-04-01

    Langerhans cell histiocytosis (LCH) has diverse clinical manifestations, including intracranial mass lesions. We report a case of LCH that manifested as a suprasellar mass, and initially misdiagnosed as a germ cell tumor. A 29-year-old woman presented with polyuria, polydipsia and amenorrhea. Laboratory findings revealed hypopituitarism with central diabetes insipidus, and a suprasellar mass and a pineal mass were observed on magnetic resonance imaging. Under the clinical impression of a germ cell tumor, the patient was treated with germ cell tumor chemotherapy (cisplatin and etoposide) and radiation therapy without biopsy. After initial shrinkage of the lesions, further growth of the tumor was observed and a biopsy was performed. The histopathology revealed LCH. After chemotherapy according to the LCH III protocol, the tumor disappeared. She is on regular follow up for 5 years without relapse. The present findings indicate that LCH should be included in the differential diagnosis of a suprasellar mass, even in adults, especially when it manifests with diabetes insipidus. This case also underscores the importance of a histopathologic diagnosis in patients with suprasellar tumors before the initiation of a specific therapy, even if the clinical findings are highly suggestive of a specific diagnosis. PMID:27195259

  4. Adult multisystem langerhans cell histiocytosis presenting with central diabetes insipidus successfully treated with chemotherapy.

    PubMed

    Choi, Jung-Eun; Lee, Hae Ri; Ohn, Jung Hun; Moon, Min Kyong; Park, Juri; Lee, Seong Jin; Choi, Moon-Gi; Yoo, Hyung Joon; Kim, Jung Han; Hong, Eun-Gyoung

    2014-09-01

    We report the rare case of an adult who was diagnosed with recurrent multisystem Langerhans cell histiocytosis (LCH) involving the pituitary stalk and lung who present with central diabetes insipidus and was successfully treated with systemic steroids and chemotherapy. A 49-year-old man visited our hospital due to symptoms of polydipsia and polyuria that started 1 month prior. Two years prior to presentation, he underwent excision of right 6th and 7th rib lesions for the osteolytic lesion and chest pain, which were later confirmed to be LCH on pathology. After admission, the water deprivation test was done and the result indicated that he had central diabetes insipidus. Sella magnetic resonance imaging showed a mass on the pituitary stalk with loss of normal bright spot at the posterior lobe of the pituitary. Multiple patchy infiltrations were detected in both lung fields by computed tomography (CT). He was diagnosed with recurrent LCH and was subsequently treated with inhaled desmopressin, systemic steroids, vinblastine, and mercaptopurine. The pituitary mass disappeared after two months and both lungs were clear on chest CT after 11 months. Although clinical remission in multisystem LCH in adults is reportedly rare, our case of adult-onset multisystem LCH was treated successfully with systemic chemotherapy using prednisolone, vinblastine, and 6-mercaptopurine, which was well tolerated. PMID:25309800

  5. Adult Multisystem Langerhans Cell Histiocytosis Presenting with Central Diabetes Insipidus Successfully Treated with Chemotherapy

    PubMed Central

    Choi, Jung-Eun; Lee, Hae Ri; Ohn, Jung Hun; Moon, Min Kyong; Park, Juri; Lee, Seong Jin; Choi, Moon-Gi; Yoo, Hyung Joon; Kim, Jung Han

    2014-01-01

    We report the rare case of an adult who was diagnosed with recurrent multisystem Langerhans cell histiocytosis (LCH) involving the pituitary stalk and lung who present with central diabetes insipidus and was successfully treated with systemic steroids and chemotherapy. A 49-year-old man visited our hospital due to symptoms of polydipsia and polyuria that started 1 month prior. Two years prior to presentation, he underwent excision of right 6th and 7th rib lesions for the osteolytic lesion and chest pain, which were later confirmed to be LCH on pathology. After admission, the water deprivation test was done and the result indicated that he had central diabetes insipidus. Sella magnetic resonance imaging showed a mass on the pituitary stalk with loss of normal bright spot at the posterior lobe of the pituitary. Multiple patchy infiltrations were detected in both lung fields by computed tomography (CT). He was diagnosed with recurrent LCH and was subsequently treated with inhaled desmopressin, systemic steroids, vinblastine, and mercaptopurine. The pituitary mass disappeared after two months and both lungs were clear on chest CT after 11 months. Although clinical remission in multisystem LCH in adults is reportedly rare, our case of adult-onset multisystem LCH was treated successfully with systemic chemotherapy using prednisolone, vinblastine, and 6-mercaptopurine, which was well tolerated. PMID:25309800

  6. Disseminated langerhans cell histiocytosis presenting as cholestatic jaundice.

    PubMed

    Kapoor, Rohit; Loizides, Anthony M; Sachdeva, Soumya; Paul, Premila

    2015-02-01

    Langerhans cell histiocytosis (LCH) is a disorder associated with proliferation of Langerhans cells in various organs. LCH secondary to multisystem involvement can present in a variety of ways. Because of its infiltrative nature, LCH can involve the skin, lymph nodes, the lung or the liver. Jaundice in LCH is a manifestation of liver disease; biliary dilatation secondary to lithiasis or may be due to coexistent Niemann-Pick disease. However, a case of cholestasis has been very rarely described. Cholestasis may result from lymph nodes obstructing the porta hepatis. In this report, we describe a case of type II histiocytosis X with obstructive cholestasis and pulmonary involvement in the form of cysts without significant lymphadenopathy at the porta. PMID:25859497

  7. Histiocytosis X: Characteristics, behavior, and treatments as illustrated in a case series

    PubMed Central

    Kasper, Ekkehard M.; Aguirre-Padilla, David H.; Alter, Raanan Y.; Anderson, Matthew

    2011-01-01

    Background: Langerhans cell histiocytosis (LCH) is a proliferative disorder predominantly found in children. It often presents with pain in calvarium or spine and may cause neuroendocrine symptoms. The gold standard for diagnosing LCH is the detection of Birbeck Granules by EM. Here, we describe two unique presentations of LCH and we review current treatment guidelines. Case Description: The first patient was a 23-year-old man who presented with progressive swelling and redness of the left eye. MRI revealed a left retrobulbar lesion extending into the middle cranial fossa with no signal abnormality in the brain parenchyma. The lesion was resected and pathological analysis revealed LCH. Bone scans were negative and the patient was discharged soon after. He later underwent fractionated radiotherapy (cumulative dose 26 Gy). Follow-up MRIs show no disease at 24 months post-op. The second patient was a 56-year-old man with left frontal skull pain for 5 months. Imaging showed a solitary osteolytic lesion extending into both dura and scalp with no signal abnormality of the parenchyma. Excisional biopsy revealed LCH. Surgery was well tolerated and follow-up imaging shows no recurrence at 24 months post-op. Conclusion: We demonstrate that LCH, though uncommon, must remain on the differential when osteolytic lesions present in the adult. Although LCH often has the clinical and radiographical presentation of an abscess, pathology analysis can successfully diagnose LCH based on markers and morphological characteristics. LCH has an excellent prognosis when treated aggressively with surgical resection and radiotherapy as both of our patients were and are now disease free at 2 year follow-up. PMID:21697965

  8. Malignant histiocytosis (histiocytic medullary reticulosis) with spindle cell differentiation and tumour formation.

    PubMed Central

    Macgillivray, J B; Duthie, J S

    1977-01-01

    Malignant histiocytosis (histiocytic medullary reticulosis) in a 45-year-old white man is described. Unusual features were presentation as a surgical emergency with signs of obstruction and peritonitis due to an ileal tumour and extensive spindle cell differentiation. Problems in the differential diagnosis of malignant histiocytosis are briefly discussed. Images PMID:845260

  9. Isolated intramedullary histiocytosis-X of the cervical spinal cord. Case report.

    PubMed

    Hamilton, B; Connolly, E S; Mitchell, W T

    1995-10-01

    Histiocytosis-X is known to involve the central nervous system, but rarely does this disease involve the spinal cord. To the authors' knowledge, this is the first case of isolated intramedullary histiocytosis-X of the spinal cord to be reported. PMID:7674022

  10. Spontaneous extradural hemorrhage due to Langerhans cell histiocytosis of the skull in a child: A rare presentation.

    PubMed

    Bakhaidar, Mohamad G; Alghamdi, Fahad A; Baeesa, Saleh S

    2016-01-01

    Eosinophilic granuloma (EG) represents a local form of Langerhans cell histiocytosis that occurs mostly in children. It usually presents with a gradually enlarging painless skull mass, and rarely presents a rapid clinical deterioration. This 7-year-old boy who was diagnosed with EG, based on a magnetic resonance imaging scan, after presenting with a painless right parietal swelling of 7-week duration. Three weeks prior his scheduled surgery, he presented to the emergency department with a 2-day history of sudden increased of the subcutaneous swelling associated with a headache, vomiting, and decreased the level of consciousness; there was no history of trauma. Brain computed tomography revealed a right parietal bone defect with large subgaleal and extradural hematoma. He underwent emergent surgical excision of the skull lesion and evacuation of the hematoma. Histopathological examination confirmed the diagnosis of EG. We aim to raise the awareness of physicians of this rare spontaneous hemorrhagic complication of EG and review the literature. PMID:27195034

  11. Spontaneous extradural hemorrhage due to Langerhans cell histiocytosis of the skull in a child: A rare presentation

    PubMed Central

    Bakhaidar, Mohamad G.; Alghamdi, Fahad A.; Baeesa, Saleh S.

    2016-01-01

    Eosinophilic granuloma (EG) represents a local form of Langerhans cell histiocytosis that occurs mostly in children. It usually presents with a gradually enlarging painless skull mass, and rarely presents a rapid clinical deterioration. This 7-year-old boy who was diagnosed with EG, based on a magnetic resonance imaging scan, after presenting with a painless right parietal swelling of 7-week duration. Three weeks prior his scheduled surgery, he presented to the emergency department with a 2-day history of sudden increased of the subcutaneous swelling associated with a headache, vomiting, and decreased the level of consciousness; there was no history of trauma. Brain computed tomography revealed a right parietal bone defect with large subgaleal and extradural hematoma. He underwent emergent surgical excision of the skull lesion and evacuation of the hematoma. Histopathological examination confirmed the diagnosis of EG. We aim to raise the awareness of physicians of this rare spontaneous hemorrhagic complication of EG and review the literature. PMID:27195034

  12. Percutaneous vertebroplasty for Langerhans cell histiocytosis of the lumbar spine in an adult: Case report and review of the literature

    PubMed Central

    FENG, FEI; TANG, HAI; CHEN, HAO; JIA, PU; BAO, LI; LI, JIN-JUN

    2013-01-01

    Langerhans cell histiocytosis (LCH) is extremely rare in the lumbar spine of adults. The radiological features typically manifest as vertebral tumors. The exact etiology of LCH remains unknown. Langerhans cells may cause local or systemic effects. The most frequent sites of these bony lesions are the skull, femur, mandible, pelvis and spine. To date, only 3 spinal LCH cases treated by percutaneous vertebroplasty (PVP) have been reported. The present study reports a case of LCH of the fourth lumbar vertebra (L4) in a 51-year-old male with a 10-day history of low back pain, limited waist motion and right lower limb numbness. The patient was treated using PVP. The use of PVP for treating LCH of the spine was successful. The present study provides an up-to-date literature overview of LCH. PMID:23251253

  13. A case of invasive Langerhans cell histiocytosis localizing only in the lung and diagnosed as pneumothorax in an adolescent female.

    PubMed

    Dejima, Hitoshi; Morita, Shigeki; Takahashi, Yusuke; Matsutani, Noriyuki; Iinuma, Hisae; Kondo, Fukuo; Kawamura, Masafumi

    2015-01-01

    In infants, Langerhans cell histiocytosis (LCH) is associated with poor clinical outcomes as Langerhans cells invade and damage multiple organs, a presentation that is different from that in adults. Here, we present a case of a 15-year-old female who visited ourclinic complaining of right chest pain and dyspnea. She was diagnosed with right pneumothorax by chest X-ray. Chest computed tomography showed multiple cystic changes in the bilateral lung. Additionally, bullous lesions occupying the upper lobe and multiple white tiny nodules on the surface of the lung were observed by thoracoscopy. These nodules comprised proliferating atypical CD1a/S-100-positive cells invading the pulmonary parenchyma, leading to the diagnosis of LCH. Because of the extensive invasion into the pulmonary parenchyma, chemotherapy was administered. This case of LCH was unique in that the age of onset was atypical and the tumor cells occupied a single organ, despite their malignant behavior. PMID:26045867

  14. A case of invasive Langerhans cell histiocytosis localizing only in the lung and diagnosed as pneumothorax in an adolescent female

    PubMed Central

    Dejima, Hitoshi; Morita, Shigeki; Takahashi, Yusuke; Matsutani, Noriyuki; Iinuma, Hisae; Kondo, Fukuo; Kawamura, Masafumi

    2015-01-01

    In infants, Langerhans cell histiocytosis (LCH) is associated with poor clinical outcomes as Langerhans cells invade and damage multiple organs, a presentation that is different from that in adults. Here, we present a case of a 15-year-old female who visited ourclinic complaining of right chest pain and dyspnea. She was diagnosed with right pneumothorax by chest X-ray. Chest computed tomography showed multiple cystic changes in the bilateral lung. Additionally, bullous lesions occupying the upper lobe and multiple white tiny nodules on the surface of the lung were observed by thoracoscopy. These nodules comprised proliferating atypical CD1a/S-100-positive cells invading the pulmonary parenchyma, leading to the diagnosis of LCH. Because of the extensive invasion into the pulmonary parenchyma, chemotherapy was administered. This case of LCH was unique in that the age of onset was atypical and the tumor cells occupied a single organ, despite their malignant behavior. PMID:26045867

  15. The orbits in cancer imaging

    PubMed Central

    Chong, V F H

    2006-01-01

    Primary malignant lesions in the orbit are relatively uncommon. However, the orbits are frequently involved in haematogeneous metastasis or by direct extension from malignancies originating from the adjacent nasal cavity or paranasal sinuses. This paper focuses on the more commonly encountered primary orbital malignancies and the mapping of tumour spread into the orbits. PMID:17114076

  16. How I treat Langerhans cell histiocytosis

    PubMed Central

    Allen, Carl E.; Ladisch, Stephan

    2015-01-01

    “Langerhans cell histiocytosis” (LCH) describes a spectrum of clinical presentations ranging from a single bone lesion or trivial skin rash to an explosive disseminated disease. Regardless of clinical severity, LCH lesions share the common histology of CD1a+/CD207+ dendritic cells with characteristic morphology among an inflammatory infiltrate. Despite historical uncertainty defining LCH as inflammatory vs neoplastic and incomplete understanding of mechanisms of pathogenesis, clinical outcomes have improved markedly over the past decades through cooperative randomized clinical trials based on empiric therapeutic strategies. Significant advances include recognition of high- and low-risk clinical groups defined by hematopoietic and/or hepatic involvement, and of the importance of optimal intensity and of duration of chemotherapy. Nevertheless, mortality of high-risk patients, disease recurrence, lack of robustly tested salvage strategies, and significant disease morbidity of both high- and low-risk patients remain challenges. Recent discovery of recurrent somatic mutations in mitogen-activated protein kinase pathway genes at critical stages of myeloid hematopoietic differentiation in LCH patients supports redefinition of the disease as a myeloproliferative disorder and provides opportunities to develop novel approaches to diagnosis and therapy. PMID:25827831

  17. Isolated Langerhans cell histiocytosis of the sublingual gland in an adult

    PubMed Central

    Yang, Shaodong; Chen, Xinming; Zhang, Jiali; Fang, Qiong

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare disorder characterized by the proliferation of pathologic Langerhans cells. Its clinical presentation is highly variable, that range from single-system, limited disease to severe, multi-organ disease with high mortality. LCH usually affects children and young adults. The most frequent sites for LCH are the bone, skin, lung, pituitary gland, and lymph nodes. Salivary gland involvement by LCH is extremely rare, and only a few cases of LHC involving the parotid glands have been reported in the English literature. To our knowledge, the involvement of the sublingual gland as a part of single or multisystem LCH has not been previously described. Herein we reported the first case of primary LCH of the sublingual gland. A 40-year-old woman presented with a 2-month history of a painless mass on the right sublingual area. Excision of the lesion including the right sublingual gland was performed. Histopathological diagnosis of LCH was rendered. The patient remains free of symptoms 17 months after surgery. PMID:26722591

  18. Isolated Langerhans cell histiocytosis of the sublingual gland in an adult.

    PubMed

    Yang, Shaodong; Chen, Xinming; Zhang, Jiali; Fang, Qiong

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare disorder characterized by the proliferation of pathologic Langerhans cells. Its clinical presentation is highly variable, that range from single-system, limited disease to severe, multi-organ disease with high mortality. LCH usually affects children and young adults. The most frequent sites for LCH are the bone, skin, lung, pituitary gland, and lymph nodes. Salivary gland involvement by LCH is extremely rare, and only a few cases of LHC involving the parotid glands have been reported in the English literature. To our knowledge, the involvement of the sublingual gland as a part of single or multisystem LCH has not been previously described. Herein we reported the first case of primary LCH of the sublingual gland. A 40-year-old woman presented with a 2-month history of a painless mass on the right sublingual area. Excision of the lesion including the right sublingual gland was performed. Histopathological diagnosis of LCH was rendered. The patient remains free of symptoms 17 months after surgery. PMID:26722591

  19. [Pulmonary Langerhans cell histiocytosis in a non-smoking Japanese woman].

    PubMed

    Tadokoro, Akira; Ishii, Tomoya; Bandoh, Shuji; Yokomise, Hiroyasu; Haba, Reiji; Ishida, Toshihiko

    2011-03-01

    A 44-year-old Japanese woman was admitted to our hospital because of dry cough and dyspnea on exertion. She had never smoked. She had been passively exposed to smoking by her husband and co-workers from the age of 21 (1984) to 33 (1996). She had previously developed pneumothorax twice, in 1985. On admission, computed tomography (CT) of the chest showed reticulonodular opacities predominant in bilateral upper lung fields, and pulmonary function tests revealed a decrease in vital capacity. The differential diagnoses were sarcoidosis, idiopathic pulmonary fibrosis and pulmonary Langerhans cell histiocytosis (PLCH). Video-assisted thoracic surgery was performed to make a definitive diagnosis. A histological specimen revealed the presence of CD1a-positive Langerhans cells in bronchiolocentric nodular lesions, leading to a diagnosis of PLCH. She was given 0.5 mg/kg bodyweight/ day oral prednisolone. Her symptoms disappeared with steroid maintenance therapy, and her vital capacity on pulmonary function testing was prevented from further deterioration. Based on the pathogenesis of PLCH, this case suggested that not only active smoking, but also passive smoking, played an important role in the development of PLCH. PMID:21485154

  20. Pulmonary Langerhans' cell granulomatosis (histiocytosis X): clinical analysis of 8 cases.

    PubMed

    Callebaut, W; Demedts, M; Verleden, G

    1998-10-01

    A detailed retrospective analysis of 8 cases has been set up to obtain an update of the clinical, radiological, diagnostic and therapeutical aspects of pulmonary Langerhans' cell granulomatosis (PLCG), previously called Histiocytosis X. This disease represents 2.8% of interstitial lung diseases (ILD) in a registration by pneumologists in Flanders. Seventy five% of our patients were active smokers. Cough, dyspnoe and constitutional symptoms were the main presenting symptoms. There was a 37.5% frequency of pneumothorax during the whole disease evolution and all these patients had to be treated with chemical or surgical pleurodesis. Spirometric pattern was variable but CO-transferfactor (TLCO) was significantly impaired in all our patients. Radiologically nodules and/or cystic lesions were found with preserved volumes. Open lung biopsy led to the diagnosis in 6 of the 8 cases. Transbronchial biopsies (TBB) were found to be not sensitive. Systemic staging in our group showed 50% of the patients to have a second focus of organ involvement. Treatment with corticosteroids and possibly immunosuppressives was without clear effect. Since 25% of our patients ultimately became candidate for lung transplantation extrapulmonary staging is prerogative and the high frequency of pleurodesis procedures in the past medical history of our patients has important implications since bilateral pleurodesis is still a contraindication for lung transplantation in some but not all institutions. PMID:9861760

  1. Multifocal Skeletal Tuberculosis Mimicking Langerhans Cell Histiocytosis in a Child: a Case Report With a Long-Term Follow-Up

    PubMed Central

    Haghighatkhah, Hamidreza; Jafroodi, Yousef; Sanei Taheri, Morteza; Pourghorban, Ramin; Sadeghian Dehkordy, Afarin

    2015-01-01

    Introduction: Multifocal skeletal tuberculosis is a rare condition that may masquerade as Langerhans cell histiocytosis, especially in children. Case Presentation: We report a case of multifocal osseous tuberculosis in a 5-year-old female patient admitted to our hospital with a complaint of low back pain but no history of respiratory symptoms or malaise. Radiological findings included vertebra plana and multiple lytic lesions in both the frontal and pelvic bones. An initial diagnosis of Langerhans cell histiocytosis was made based on imaging findings; however, the patient underwent further evaluation for Mycobacterium tuberculosis, and histopathologic findings confirmed the diagnosis of tuberculosis. The patient showed a nearly complete response after receiving a course of anti-tuberculosis drugs. Conclusions: A high index of suspicion is required for the early diagnosis and prompt treatment of patients with osseous tuberculosis. Given the high prevalence of tuberculosis in developing countries, tuberculosis should be considered in the differential diagnosis of multifocal lytic lesions and vertebra plana, especially in children. PMID:26744631

  2. Langerhans cell histiocytosis with nail changes and multisystem disease: a case report.

    PubMed

    De Jesus Semblano Bittencourt, Maraya; Moraes Dias, Carolina; Lima Lage, Thaiane; Magno Parijós, Amanda; Brito Mesquita, Letícia; Haber Carvalho, Alessandra

    2016-01-01

    Nail involvement in Langerhans cell histiocytosis is uncommon and is said to indicate a poor prognosis. We describe a 2-year-old boy with onycholysis, subungual hyperkeratosis, and hemorrhages on his fingernails. He also had hepatosplenomegaly and pulmonary involvement. The diagnosis of Langerhans cell histiocytosis was made by histopathologic examination of skin and liver.The role of nail involvement as an unfavorable prognostic sign is still unclear and this paper concludes that nail involvement in Langerhans cell histiocytosis is a possible sign of multisystemic involvement. PMID:27617727

  3. [Langerhans cell histiocytosis presenting as isolated adenitis in an infant: case report].

    PubMed

    Soriano-Ramos, María; Salcedo Lobato, Enrique; Baro Fernández, María; Blázquez-Gamero, Daniel

    2016-08-01

    Langerhans cell histiocytosis in infants is a rare condition, and presentation as an isolated cervical adenitis is exceptional at this age. We describe the case of a 3-month-old female infant presenting with a neck mass in the right mandibular angle with poor response to antibiotic treatment. Fine needle aspiration was performed and confirmed the diagnosis of Langerhans cell histiocytosis with complementary tests showing no features of systemic involvement. Langerhans cell histiocytosis should be considered in the differential diagnosis of subacute neck masses with poor outcome in infants and physicians should consider performing a fine needle aspiration to establish the diagnosis. PMID:27399030

  4. Different manifestations of langerhans cell histiocytosis affecting two members of a family.

    PubMed

    Balikó, Z; Schreiner, M; Kishindy, K K; Hegedüs, G; Kosztolányi, G

    2000-01-01

    Two different manifestations of Langerhans cell histiocytosis (histiocytosis X) are presented, the first occurring as a solitary rib eosinophil granuloma in an 11-year-old girl, and the second as an eosinophil granuloma of the lung of her mother, a 41-year-old heavy smoker, appearing 8 years after the onset of her daughter's disease. Familial clustering of two different manifestations of histiocytosis X disease is very rare and raises the possibility of inherited mutations that promote emergence of clonal Langerhans cells. PMID:11070469

  5. Langerhans Cell Histiocytosis and Other Histiocytic Diseases of the Lung.

    PubMed

    DeMartino, Erin; Go, Ronald S; Vassallo, Robert

    2016-09-01

    Several histiocytic disorders may affect the thoracic cavity. Pulmonary Langerhans cell histiocytosis (LCH) is the histiocytic disorder that most frequently manifests with thoracic involvement in adults and is associated with cigarette smoking. The histiocytic disorders follow variable clinical courses ranging from benign disease to life-threatening aggressive disorders. Although the pathogenesis is not fully understood, it is now apparent that some of these disorders are associated with activating mutations in cell proliferative/regulatory pathways. Management of these rare disorders must be individualized. Pharmacologic treatment may include the use of chemotherapeutic agents. Smoking cessation is imperative in the management of pulmonary LCH. PMID:27514589

  6. Intraorbital Cystic Lesions: An Imaging Spectrum.

    PubMed

    Pahwa, Shivani; Sharma, Sanjay; Das, Chandan J; Dhamija, Ekta; Agrawal, Saurabh

    2015-01-01

    Presence of a cyst or a cystic component in an intraorbital mass often narrows the list of differential diagnoses to specific entities. Such a lesion in the orbit may arise from structures within the orbit, globe, and lacrimal system or from neighboring paranasal sinuses or meninges. Common congenital and developmental lesions encountered within the orbit include dermoids and epidermoids, and infrequently coloboma. Parasitic cysts (cysticercus), orbital abscess, mucocele, and vascular lesions are the most common acquired pathologies giving rise to fluid-containing lesions within the orbit. The role of a radiologist is crucial in expediting the diagnosis of orbital lesions with the help of characteristic imaging features on ultrasound, computed tomography, or magnetic resonance imaging. It also helps in identifying complications in others where formulation of an early and effective management strategy is vital for preserving vision. PMID:25908230

  7. Treatment of localized Langerhans' cell histiocytosis of the mandible with intralesional steroid injection: report of a case.

    PubMed

    Esen, Alparslan; Dolanmaz, Doğan; Kalayci, Abdullah; Günhan, Omer; Avunduk, Mustafa Cihat

    2010-02-01

    Localized Langerhans cell histiocytosis (LLCH), formerly known as eosinophilic granuloma, mainly affects the skull, mandible, vertebrae, and ribs in children and the long bones of adults. Symptoms range from none to pain, swelling, and tenderness over the site of the lesion. General malaise and fever occasionally are present. Radiographically, lesions appear as radiolucent areas with well demarcated borders. LLCH may resolve spontaneously after biopsy in a period of months to years. However, if features include continuous pain, decrease of function, pathologic fractures, migration and resorption of teeth, or rapid progression, then active treatment needs to be considered. Treatment approaches include surgery, radiotherapy, chemotherapy, and intralesional injection of corticosteroids. In children with mandibular LLCH, 1 dose of methyprednisolone succinate injection has proven to be adequate. However, injections have not been performed in cases involving pathologic fracture. We report a new case of LLCH of the mandible that caused a pathologic fracture in an adult patient. Repeated intralesional corticosteroid injections resulted in fracture line disappearance within 14 months and lesion healing by the end of the 36-month follow-up. PMID:20123398

  8. Multi-focal histiocytosis X of bone in two adjacent vertebrae causing paraplegia.

    PubMed

    Turgut, M; Gurçay, O

    1992-03-01

    This report describes a case of multi-focal histiocytosis X of bone in two adjacent vertebrae that caused a spinal cord compression. This case was treated radically with combined surgery and postoperative radiotherapy (RT). PMID:1550511

  9. Redefining Langerhans Cell Histiocytosis as a Myeloid Dysplasia and Identifying B | Division of Cancer Prevention

    Cancer.gov

    DESCRIPTION (provided by applicant): Redefining Langerhans Cell Histiocytosis as a Myeloid Dysplasia and Identifying Biomarkers for Early Detection and Risk Assessment. This application addresses Program Announcement PA-09-197: Biomarkers for Early Detection of Hematopoietic Malignancies (R01). The overall aim of this project is to identify novel biomarkers that may be used to diagnose and treat patients with Langerhans Cell Histiocytosis (LCH). LCH occurs with similar frequency as other rare malignancies including Hodgkin's lymphoma and AML. |

  10. Bone marrow infiltration in Langerhan’s cell histiocytosis - An unusual but important determinant for staging and treatment

    PubMed Central

    Kumar, Mahendra; Updesh Singh Sachdeva, Man; Naseem, Shano; Ahluwalia, Jasmina; Das, Reena; Varma, Neelam; Marwaha, R K

    2015-01-01

    Background: Langerhans' cell histiocytosis (LCH) is a reactive proliferative disease of unknown pathogenesis characterized by proliferation of Langerhans cells. Involvement of bone marrow (BM), liver and lung are related to high risk factors and poor survival. The aim of this report is to highlight the clinical and haematological findings of 5 cases of LCH with BM infiltration which may help to predict involvement of BM. Case series: Five cases of Langerhan’s cell histiocytosis with bone marrow infiltration were retrieved from archives of Department of Hematology, PGIMER and Chandigarh for review and further analysis. Male to female ratio was 3:2 with mean age of 9.4 months. Two out of 5 patients had obvious skull swelling; however, radiography of the skull revealed lytic lesion of skull in 4 cases and 2 had skin rashes. Hepatomegaly was present in 4 cases and 2 of whom also had lymphadenopathy and splenomegaly. All patients had anaemia at the time of presentation. Bone marrow aspiration and trephine biopsy in all 5 cases revealed infiltration by large histiocytes with abundant cytoplasm and coffee bean shaped nucleus. Nodules of these Langerhans cells with admixture of eosinophils were seen on trephine biopsy. Immunohistochemistry showed positivity for CD1a stain. Conclusion: BM evaluation is important in LCH patients to categorize disease which further determines the type of therapy to be given. Clinical details may help to predict the BM involvement; however, demonstration of CD1a positive cells in marrow is most important tool to diagnose marrow infiltration by LCH. PMID:26865930

  11. Cytological diagnosis of Langerhans cell histiocytosis with cutaneous involvement.

    PubMed

    Chandekar, Sushama A; Shah, Vinaya B; Kavishwar, Vikas

    2013-01-01

    Langerhans cell histiocytosis (LCH) is a rare disease affecting predominantly children. The course of the disease varies, from spontaneous resolution to a progressive multisystem disorder with organ dysfunction and potential life-threatening complications. Diagnosis of LCH is often difficult and may be delayed because of its rarity and especially so if it occurs with unusual presentation. Fine needle aspiration cytology of a 4 year old male child, a case of LCH is presented with a purpose of highlighting the characteristic cytological features. A high index of suspicion, awareness of characteristic cytological features of LCH and its differential diagnoses is necessary. This can obviate the need of biopsy and electron microscopy. Immunohistochemistry if available can be performed on cytology smear and cell block. PMID:23661952

  12. Solitary pulmonary MALT lymphoma presenting crystal-storing histiocytosis.

    PubMed

    Nagaharu, Keiki; Kageyama, Yuki; Watanabe, Takuya; Yamaguchi, Takanori; Ito, Ryugo; Baba, Youichirou; Masuya, Masahiro; Ohashi, Riuko; Kawakami, Keiki

    2016-08-01

    Crystal-storing histiocytosis (CSH) is characterized by the accumulation of large histiocytes with intracytoplasmic crystallized immunoglobulin and is typically associated with hematological malignancies. A 69-year-old man, who had a history of left nephrectomy and chemotherapy for renal pelvic cancer six years earlier, had received a CT scan every year thereafter and a small nodule was found in the left lower lobe of his lungs two years prior to the current presentation. Because of progression of this pulmonary nodule, he underwent pulmonary lobectomy on suspicion of lung cancer. He was ultimately diagnosed as having CSH accompanied by mucosa-associated lymphoid tissue lymphoma stage IAE. In the absence of further treatment, he has been well with no recurrence of the disease for 10 months postoperatively. Because CSH could reportedly be an initial presentation of hematological malignancies, careful observation and evaluation for the presence of these blood disorders is essential. PMID:27599420

  13. Sinus histiocytosis with massive lymphadenopathy: a "massive" misnomer.

    PubMed

    Sardana, Divesh; Goyal, Ashima; Gauba, Krishan

    2015-04-01

    Sinus histiocytosis with massive lymphadenopathy also known as Rosai-Dorfman disease is a rare, benign, histiocytic disorder of unknown origin characterized by lymphadenopathy. Since its original description by Rosai and Dorfman in 1969, small number of cases has been reported; hence no specific diagnostic criteria and treatment guidelines have been suggested. The purpose of this article is to present and discuss a case of Rosai Dorfman Syndrome in 4-year-old child diagnosed with the help of Fine Needle Aspiration Cytology. The swelling resolved over a period of 18 months without any recurrence in 2-year follow-up period. This case report highlights the role of Fine Needle Aspiration Cytology in the diagnosis of Rosai-Dorfman disease and the importance of regular follow ups using a careful wait and watch approach in its management. The report also briefly discusses the various dilemmas associated with its diagnosis and treatment. PMID:25088489

  14. Langerhans cell histiocytosis causing cervical myelopathy in a child.

    PubMed

    Jang, Kun Soo; Jung, Youn Young; Kim, Seok Won

    2010-06-01

    Langerhans cell histiocytosis (LCH), a disorder of the phagocytic system, is a rare condition. Moreover, spinal involvement causing myelopathy is even rare and unusual. Here, we report a case of atypical LCH causing myelopathy, which was subsequently treated by corpectemy and fusion. A 5-year-old boy presented with 3 weeks of severe neck pain and limited neck movement accompanying right arm motor weakness. CT scans revealed destruction of C7 body and magnetic resonance imaging showed a tumoral process at C7 with cord compression. Interbody fusion using cervical mesh packed by autologus iliac bone was performed. Pathological examination confirmed the diagnosis of LCH. After the surgery, the boy recovered from radiating pain and motor weakness of right arm. Despite the rarity of the LCH in the cervical spine, it is necessary to maintain our awareness of this condition. When neurologic deficits are present, operative treatment should be considered. PMID:20617093

  15. Langerhans Cell Histiocytosis Causing Cervical Myelopathy in a Child

    PubMed Central

    Jang, Kun Soo; Jung, Youn Young

    2010-01-01

    Langerhans cell histiocytosis (LCH), a disorder of the phagocytic system, is a rare condition. Moreover, spinal involvement causing myelopathy is even rare and unusual. Here, we report a case of atypical LCH causing myelopathy, which was subsequently treated by corpectemy and fusion. A 5-year-old boy presented with 3 weeks of severe neck pain and limited neck movement accompanying right arm motor weakness. CT scans revealed destruction of C7 body and magnetic resonance imaging showed a tumoral process at C7 with cord compression. Interbody fusion using cervical mesh packed by autologus iliac bone was performed. Pathological examination confirmed the diagnosis of LCH. After the surgery, the boy recovered from radiating pain and motor weakness of right arm. Despite the rarity of the LCH in the cervical spine, it is necessary to maintain our awareness of this condition. When neurologic deficits are present, operative treatment should be considered. PMID:20617093

  16. Cytological diagnosis of Langerhans cell histiocytosis with cutaneous involvement

    PubMed Central

    Chandekar, Sushama A; Shah, Vinaya B; Kavishwar, Vikas

    2013-01-01

    Langerhans cell histiocytosis (LCH) is a rare disease affecting predominantly children. The course of the disease varies, from spontaneous resolution to a progressive multisystem disorder with organ dysfunction and potential life-threatening complications. Diagnosis of LCH is often difficult and may be delayed because of its rarity and especially so if it occurs with unusual presentation. Fine needle aspiration cytology of a 4 year old male child, a case of LCH is presented with a purpose of highlighting the characteristic cytological features. A high index of suspicion, awareness of characteristic cytological features of LCH and its differential diagnoses is necessary. This can obviate the need of biopsy and electron microscopy. Immunohistochemistry if available can be performed on cytology smear and cell block. PMID:23661952

  17. Adult langerhans cell histiocytosis with hepatic and pulmonary involvement.

    PubMed

    Araujo, Bruno; Costa, Francisco; Lopes, Joanne; Castro, Ricardo

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of Langerhans cells of unknown etiology. It can involve multiple organ systems with different clinical presentation, which complicates the diagnosis. It can range from isolated to multisystem disease with different prognosis. Although common among children, liver involvement is relatively rare in adults and frequently overlooked. Natural history of liver LCH fits into two stages: an early stage with infiltration by histiocytes and a late stage with sclerosis of the biliary tree. Pulmonary findings are more common and include multiple nodules in different stages of cavitation, predominantly in the upper lobes. We present a case of adult LCH with pulmonary and biopsy proven liver involvement with resolution of the hepatic findings after treatment. PMID:25977828

  18. Utility of spiral CAT scan in the follow-up of patients with pulmonary Langerhans cell histiocytosis.

    PubMed

    Abbritti, M; Mazzei, M A; Bargagli, E; Refini, R M; Penza, F; Perari, M G; Volterrani, L; Rottoli, P

    2012-08-01

    Pulmonary Langerhans cell histiocytosis (PLCH) is a rare disease that occurs almost exclusively in smokers, generally young adults between 20 and 40 years old. Prognostic biomarkers of the disease are lacking. This study describes the clinical-radiological features of a group of PLCH patients and applies a semi-quantitative CT score of the chest to verify the prognostic value of radiological findings in this disease. Clinical-radiological and immunological data from 12 Caucasian patients (6M, 7 smokers and 5 ex-smokers, mean age 36±8 years) were recorded at onset and after a follow-up period of 4 years. Application of the semi-quantitative CT score revealed a prevalently cystic pattern at onset and follow-up in the majority of the patients. Patients with a prevalently nodular pattern developed cystic lesions during follow-up. Interestingly, significant correlations were found between the extent of cystic lesions and DLCO values at onset (time 0: p<0.05) and at the end of follow-up (time 1, p<0.05) and with FEV1 values at time 0 (p<0.05) and time 1 (p<0.05). Patients with progressive functional decline were those with CT evidence of severe cystic alterations. The results suggest that high resolution CT scan of the chest is mandatory for characterizing PLCH patients at diagnosis and during follow-up. The proposed CT score of the chest showed potential prognostic value. PMID:21549535

  19. Central nervous system imaging in childhood Langerhans cell histiocytosis – a reference center analysis

    PubMed Central

    Porto, Luciana; Schöning, Stefan; Hattingen, Elke; Sörensen, Jan; Jurcoane, Alina; Lehrnbecher, Thomas

    2015-01-01

    Background The aim of our study was (1) to describe central nervous system (CNS) manifestations in children with Langerhans cell histiocytosis (LCH) based on images sent to a reference center and meeting minimum requirements and (2) to assess the inter-rater agreement of CNS-MRI results, which represents the overall reproducibility of this investigation. Methods We retrospectively reviewed brain MRI examinations in children with LCH, for which MRI minimum requirements were met. Abnormalities were rated by two experienced neuroradiologists, and the inter-rater agreement was assessed. Results Out of a total of 94 imaging studies, only 31 MRIs met the minimum criteria, which included T2w, FLAIR, T1w images before/after contrast in at least two different section planes, and thin post contrast sagittal slices T1w through the sella. The most common changes were osseous abnormalities, followed by solid enlargement of the pineal gland, thickened enhancing stalk and signal changes of the dentate nucleus. Whereas inter-rater agreement in assessing most of the CNS lesions was relatively high (κ > 0.61), the application of minimum criteria often did not allow to evaluate the posterior pituitary. Conclusions The diversity of radiological protocols from different institutions leads to difficulties in the diagnosis of CNS abnormalities in children with LCH. Although the inter-rater agreement between neuroradiologists was high, not all the LCH manifestations could be completely ruled out when using the minimum criteria. Brain MRIs should therefore follow LCH guideline protocols and include T1 pre-gadolinium sagittal images, and be centrally reviewed in order to improve the comparison of clinical trials. PMID:26401129

  20. Langerhans cell histiocytosis in children: from the bench to bedside for an updated therapy.

    PubMed

    Aricò, Maurizio

    2016-06-01

    Langerhans cell histiocytosis (LCH) is a rare disease, affecting subjects of any age, with extremely variable clinical manifestations. Although most patients with LCH have localized disease, requiring local or even no therapy, those patients with disseminated, 'multi-system' disease require specific therapy because they may be at risk for morbidity or even mortality. The current standard of care has developed empirically, based mainly on the experience of treating children with leukaemia and other haemo-proliferative disorders. At the time of writing, the combined use of vinblastine and prednisone remains the standard of care for children with multi-system LCH. The combination of cytarabine and cladribine is the current standard for second-line therapy of refractory cases with vital organ dysfunction. Recent advances in the knowledge of the pathogenesis of LCH may support a change in treatment strategy. Evidence of mutations that aberrantly activate RAF/MEK/ERK signalling in over two thirds of patients with LCH may direct a target therapy strategy. Vemurafenib, a small molecule widely used in the treatment of melanoma, is the main candidate for testing in prospective trials for patients with evidence of BRAF(V) (600E) mutation on lesional tissue. Additional molecules, including the recently approved trametinib, could follow. Identification of mutations in other genes in the remaining multisystem LCH cases could contribute to define a scenario in which target therapy becomes the main therapeutic choice in this intriguing disorder. However, because the long-term risks and benefits of these agents in children are unknown, and other effective treatments exist for many LCH patients, the optimal indications for administering a tyrosine kinase inhibitor to children is an open question. PMID:26913480

  1. BRAF and MAP2K1 mutations in Langerhans cell histiocytosis: a study of 50 cases.

    PubMed

    Alayed, Khaled; Medeiros, L Jeffrey; Patel, Keyur P; Zuo, Zhuang; Li, Shaoying; Verma, Shalini; Galbincea, John; Cason, R Craig; Luthra, Rajyalakshmi; Yin, C Cameron

    2016-06-01

    Langerhans cell histiocytosis (LCH) is a proliferation of Langerhans cells, often associated with lymphocytes, eosinophils, macrophages, and giant cells. BRAF mutations, usually V600E, have been reported in 40%-70% of cases, and recently, MAP2K1 mutations have been reported in BRAF-negative cases. We assessed 50 cases of LCH for BRAF mutations and assessed a subset of cases for MAP2K1 mutations. The study group included 28 men and 22 women (median age, 36.5 years; range, 1-78 years). BRAF V600E mutation was detected in 8 (16%) cases including 3 (30%) skin, 2 (11%) bone, 1 (50%) colon, 1 (20%) lung, and 1 (33%) extradural, intracranial mass. MAP2K1 mutations were detected in 6 of 13 (46%) BRAF-negative cases including 2 (100%) lymph node, 2 (50%) bone, 1 (25%) skin, and 1 (100%) orbit. Patients with BRAF mutation were younger than patients with wild-type BRAF (median age, 28 versus 38 years; P = .026). The median age of MAP2K1-mutated patients was 34.5 years, similar to patients without MAP2K1 mutation (41 years; P = .368). In agreement with 2 recent studies, we showed a high frequency of MAP2K1 mutations in BRAF-negative LCH cases. Unlike other studies, the overall frequency of BRAF mutation in this cohort is substantially lower than what has been reported in pediatric patients, perhaps because most patients in this study were adults. Moreover, we showed a high concordance between mutational and immunohistochemical analysis for BRAF mutation. There was no statistically significant association between BRAF or MAP2K1 mutation and anatomic site, unifocal versus multifocal presentation, or clinical outcome. PMID:26980021

  2. [Anesthetic Management of a Patient with Langerhans Cell Histiocytosis during Cesarean section].

    PubMed

    Ishii, Erika; Takaenoki, Yumiko; Shizukuishi, Masaaki; Fukuda, Isao; Kazama, Tomiei

    2015-04-01

    Langerhans cell histiocytosis is a rare disease, associated with histiocyte increases, and granuloma, in various organs. About 160 patients are reported in Japan. A pregnant patient with a pulmonary Langerhans cell histiocytosis underwent cesarean section under spinal anesthesia. She had repeated pneumothorax with bilateral pulmonary cysts rapidly becoming worse during pregnancy. She was treated with continuous oxygen after 28 weeks of the pregnancy. On 34 weeks of the pregnancy, spinal anesthesia with 0.5% hyperbaric bupivacaine (2 ml) and fentanyl (25 μg) for cesarean section was performed, and provided excellent analgesia without any side-effects. PMID:26419115

  3. Pulmonary langerhans cell histiocytosis: PET/CT for initial workup and treatment response evaluation.

    PubMed

    Hansen, Neil J; Hankins, Jordan H

    2015-02-01

    A 40-year-old man underwent pan-endoscopy owing to abdominal pain. Biopsies of the gastrointestinal tract demonstrated diffuse Langerhans cell histiocytosis. PET/CT was done, with CT demonstrating classic pulmonary manifestations of Langerhans cell histiocytosis that had association with intense FDG uptake on PET. Bowel appeared normal. Treatment was initiated with smoking cessation and 6 cycles of cytarabine. Follow-up PET/CT after initial treatment demonstrated improvement of parenchymal abnormalities seen on CT, with resolution of hypermetabolic activity. Maintenance chemotherapy was initiated. PET/CT is increasingly being used for initial staging and treatment response assessment in this rare disorder. PMID:24999688

  4. A case of Langerhans' cell histiocytosis following Hodgkin's disease

    PubMed Central

    LI, XIN; DENG, QI; LI, YU-MING

    2016-01-01

    Langerhans' cell histiocytosis (LCH) is a group of disorders in various tissues characterized by the proliferation of Langerhans cells. It is rarely observed in adults. Langerhans cells are dendritic cells that express cluster of differentiation 1a (CD1a) and S100 protein, and contain Birbeck granules. Its etiopathogenesis remains to be elucidated. One possible etiological cause is a reactive proliferation of Langerhans cells following chemotherapy or radiotherapy for Hodgkin's disease (HD). A number of cases of LCH associated with malignant lymphoma have been reported previously. It may follow after the malignant lymphoma, or occur with it. However, fewer cases have been reported where the LCH followed after HD. In the present case report, a patient was diagnosed with HD following chemotherapy for LCH. As LCH was diagnosed, the patient was treated with a combination of various chemotherapeutic agents in two cycles of cyclophosphamide, vincristine, and prednisolone (COP), and eight cycles of cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP). The patient went into a successful clinical remission. One year later, computed tomographic (CT) scans of the thorax and abdomen revealed augmentation of the tumor mass in the mediastinum. An excisional biopsy of the right inguinal lymph node was performed. The patient was diagnosed with nodular sclerosing Hodgkin's disease. Following four cycles of doxorubicin (Adriamycin), bleomycin, vinblastine and dacarbazine (ABVD) chemotherapy, a whole-body positron emission tomographic CT scan revealed a decrease in tumor mass in the mediastinum. At present, the patient remains in treatment, and the prognosis has yet to be fully determined. PMID:27330759

  5. Endocrine disorders in pediatric - onset Langerhans Cell Histiocytosis.

    PubMed

    Amato, M C M; Elias, L L K; Elias, J; Santos, A C D; Bellucci, A D; Moreira, A C; De Castro, M

    2006-11-01

    Langerhans Cell Histiocytosis (LCH) is a rare disorder with a great variety of clinical manifestations. The purpose of this retrospective study was to evaluate the pattern and the long-term course of clinical, laboratorial and radiological findings in pediatric-onset LCH. We reviewed 46 children with histological diagnosis of LCH. Ten children (22%) showed endocrine disorders. Central diabetes insipidus (DI) was observed in all ten patients; GH deficiency was confirmed in four and hypogonadism in two children. There were no adrenal, prolactin or thyroid axis abnormalities. Obesity was observed in three patients. Eight patients showed soft tissue infiltration and five bone involvement. The MRI showed a lack of posterior pituitary bright spot in all DI patients; infundibular infiltration (II) associated or not with sellar or supra-sellar mass was observed in 4 patients. We conclude that the investigation of LCH, a multi-systemic disease, should include central nervous system images. The presence of II and/or DI should raise the diagnosis of LCH. Complete endocrine evaluation, allowing an early hormone therapy, is required to obtain a better quality of life in children with LCH. PMID:17111302

  6. Macrophages in Langerhans cell histiocytosis are differentiated toward M2 phenotype: their possible involvement in pathological processes.

    PubMed

    Ohnishi, Koji; Komohara, Yoshihiro; Sakashita, Naomi; Iyama, Ken-Ichi; Murayama, Toshihiko; Takeya, Motohiro

    2010-01-01

    Although numerous macrophages are found in the lesions of Langerhans cell histiocytosis (LCH), their activation phenotypes and their roles in the disease process have not been clarified. Paraffin-embedded LCH samples were examined on immunohistochemistry and it was found that CD163 can be used to distinguish infiltrated macrophages from neoplastic Langerhans cells (LC). The number of CD163-positve macrophages was positively correlated with the number of multinucleated giant cells (MGC), indicating that most MGC are derived from infiltrated macrophages. A significant number of CD163-positive macrophages were positive for interleukin (IL)-10 and phospho-signal transducer and activator of transcription-3 (pSTAT3), an IL-10-induced signal transduction molecule. This indicates that these macrophages are polarized to anti-inflammatory macrophages of M2 phenotype. Tumor-derived macrophage-colony-stimulating factor (M-CSF) was considered to responsible for inducing M2 differentiation of infiltrated macrophages. The number of CD163-positive macrophages in different cases of LCH varied, and interestingly the density of CD163-positive macrophages was inversely correlated with the Ki-67-positivity of LC. Although the underlying mechanism is not fully elucidated, macrophage-derived IL-10 was considered to be involved in the suppression of tumor cell proliferation via activation of STAT3. PMID:20055949

  7. Imaging of orbital disorders.

    PubMed

    Cunnane, Mary Beth; Curtin, Hugh David

    2016-01-01

    Diseases of the orbit can be categorized in many ways, but in this chapter we shall group them according to etiology. Inflammatory diseases of the orbits may be infectious or noninfectious. Of the infections, orbital cellulitis is the most common and typically arises as a complication of acute sinusitis. Of the noninfectious, inflammatory conditions, thyroid orbitopathy is the most common and results in enlargement of the extraocular muscles and proliferation of the orbital fat. Idiopathic orbital inflammatory syndrome is another cause of inflammation in the orbit, which may mimic thyroid orbitopathy or even neoplasm, but typically presents with pain. Masses in the orbit may be benign or malignant and the differential diagnosis primarily depends on the location of the mass lesion, and on the age of the patient. Lacrimal gland tumors may be lymphomas or epithelial lesions of salivary origin. Extraocular muscle tumors may represent lymphoma or metastases. Tumors of the intraconal fat are often benign, typically hemangiomas or schwannomas. Finally, globe tumors may be retinoblastomas (in children), or choroidal melanomas or metastases in adults. PMID:27432687

  8. Endoscopic treatment of orbital tumors

    PubMed Central

    Signorelli, Francesco; Anile, Carmelo; Rigante, Mario; Paludetti, Gaetano; Pompucci, Angelo; Mangiola, Annunziato

    2015-01-01

    Different orbital and transcranial approaches are performed in order to manage orbital tumors, depending on the location and size of the lesion within the orbit. These approaches provide a satisfactory view of the superior and lateral aspects of the orbit and the optic canal but involve risks associated with their invasiveness because they require significant displacement of orbital structures. In addition, external approaches to intraconal lesions may also require deinsertion of extraocular muscles, with subsequent impact on extraocular mobility. Recently, minimally invasive techniques have been proposed as valid alternative to external approaches for selected orbital lesions. Among them, transnasal endoscopic approaches, “pure” or combined with external approaches, have been reported, especially for intraconal lesions located inferiorly and medially to the optic nerve. The avoidance of muscle detachment and the shortness of the surgical intraorbital trajectory makes endoscopic approach less invasive, thus minimizing tissue damage. Endoscopic surgery decreases the recovery time and improves the cosmetic outcome not requiring skin incisions. The purpose of this study is to review and discuss the current surgical techniques for orbital tumors removal, focusing on endoscopic approaches to the orbit and outlining the key anatomic principles to follow for safe tumor resection. PMID:25789299

  9. The cognitive spectrum in neurodegenerative Langerhans cell histiocytosis.

    PubMed

    Le Guennec, Loïc; Decaix, Caroline; Donadieu, Jean; Santiago-Ribeiro, Maria; Martin-Duverneuil, Nadine; Levy, Richard; Delgadillo, Daniel; Kas, Aurélie; Drier, Aurélie; Magy, Laurent; Bayen, Eleonore; Hoang-Xuan, Khe; Idbaih, Ahmed

    2014-08-01

    Clinical spectrum of cognitive troubles complicating neurodegenerative Langerhans cell histiocytosis (ND-LCH) is poorly known. The aim of this study is to evaluate cognitive functions in ND-LCH. The cognitive functions of a series of eight adult patients (7 males and 1 female; mean age 26 years IQ 25-75; range 20-33) suffering from clinical and/or radiological ND-LCH were evaluated using the following tests: (1) forward/backward digit and spatial span tasks of the WAIS-R scale and the Corsi block task, (2) the French version of the free and cued selective reminding test, (3) verbal fluency tests, (4) the Frontal Assessment Battery (FAB), (5) backward measurement of the verbal and visuospatial memories of the WAIS-R scale, (6) the Rey complex figure test, (7) the trail making tests A and B, (8) digit symbol and symbol search of the WAIS-IV scale, and (9) the Stroop test. Episodic (i.e. autobiographical or personal) memory free recall, categorical verbal fluency, phonological verbal fluency, visuospatial processing skills, attention, speed of processing, and sensitivity to interference were impaired in ND-LCH patients. In contrast, verbal and visuospatial short-term memories (i.e. immediate memories or forward span tasks) were preserved in all patients. Adult ND-LCH patients suffer from a severe but dissociated dysexecutive syndrome, mostly affecting executive strategies and relatively sparing short-term memory. Our study supports the need of assessing executive functions using comprehensive cognitive evaluation in ND-LCH patients for early diagnosis. PMID:24848633

  10. Differentiating skin-limited and multisystem Langerhans cell histiocytosis

    PubMed Central

    Simko, Stephen J.; Garmezy, Benjamin; Abhyankar, Harshal; Lupo, Philip J.; Chakraborty, Rikhia; Lim, Karen Phaik Har; Shih, Albert; Hicks, M. John; Wright, Teresa S.; Levy, Moise L.; McClain, Kenneth L.; Allen, Carl E.

    2014-01-01

    Objective To identify features associated with multisystem involvement and therapeutic failure in patients with skin Langerhans cell histiocytosis (LCH). Study design We reviewed medical records of 71 consecutive LCH patients with skin involvement evaluated at Texas Children’s Hospital and analyzed clinical features, laboratory results, and presence of circulating cells with the BRAF-V600E mutation, with respect to initial staging and clinical outcomes. Results Skin disease in patients older than 18 months at diagnosis was associated with presence of multisystem disease (OR 9.65, 95% CI 1.17–79.4). Forty percent of patients referred for presumed skin-limited LCH had underlying multisystem involvement, half of these with risk-organ involvement. Patients with skin-limited LCH had 3-year progression-free survival (PFS) of 89% after initial therapy, and none developed multisystem disease. Patients with skin/multisystem involvement had 3 year PFS of 44% with vinblastine/prednisone therapy, and risk-organ involvement did not correlate with failure to achieve non-active disease. Circulating cells with BRAF-V600E were detected at higher frequency in multisystem patients (8/11 skin/multisystem, 1/13 skin-limited, P=0.002). Conclusions Skin-limited LCH requires infrequent therapeutic intervention and has lower risk of progression relative to skin plus multisystem LCH. The less aggressive clinical course and lack of circulating cells with BRAF-V600E mutation in skin-limited LCH suggest a different mechanism of disease origin compared with multisystem or risk-organ disease. PMID:25441388

  11. Vascular Lesions.

    PubMed

    Jahnke, Marla N

    2016-08-01

    Vascular lesions in childhood are comprised of vascular tumors and vascular malformations. Vascular tumors encompass neoplasms of the vascular system, of which infantile hemangiomas (IHs) are the most common. Vascular malformations, on the other hand, consist of lesions due to anomalous development of the vascular system, including the capillary, venous, arterial, and lymphatic systems. Capillary malformations represent the most frequent type of vascular malformation. IHs and vascular malformations tend to follow relatively predictable growth patterns in that IHs grow then involute during early childhood, whereas vascular malformations tend to exhibit little change. Both vascular tumors and vascular malformations can demonstrate a wide range of severity and potential associated complications necessitating specialist intervention when appropriate. Evaluation and treatment of the most common types of vascular lesions are discussed in this article. [Pediatr Ann. 2016;45(8):e299-e305.]. PMID:27517358

  12. Cluster headache after orbital exenteration.

    PubMed

    Evers, S; Sörös, P; Brilla, R; Gerding, H; Husstedt, I W

    1997-10-01

    A 37-year-old man developed an ipsilateral headache which fulfilled the criteria for cluster headache after orbital extenteration because of a traumatic lesion of the bulb. The headache could be treated successfully by drugs usually applied in the therapy of cluster headache. Six similar cases of cluster headache after orbital exenteration could be identified in the literature suggesting that the eye itself is not necessarily part of the pathogenesis of cluster headache. We hypothesize that orbital exenteration can cause cluster headache by lesions of sympathetic structures. Possibly, these mechanisms are similar to those of sympathetic reflex dystrophy (Sudeck-Leriche syndrome) causing pain of the limbs. PMID:9350391

  13. Cystic and Cavitary Lung Lesions in Children: Radiologic Findings with Pathologic Correlation

    PubMed Central

    Odev, Kemal; Guler, İbrahim; Altinok, Tamer; Pekcan, Sevgi; Batur, Abdussamed; Ozbiner, Hüseyin

    2013-01-01

    A number of diseases produce focal or multiple thin-walled or thick-walled air- or fluid-containing cysts or cavitary lung lesions in both infants and children. In infants and children, there is a spectrum of focal or multifocal cystic and cavitary lung lesions including congenital lobar emphysema, congenital cystic adenomatoid malformation, pleuropulmonary blastoma, bronchogenic cyst, pulmonary sequestration, Langerhans cell histiocytosis, airway diseases, infectious diseases (bacterial infection, fungal infection, etc.), hydatid cysts, destroid lung, and traumatic pseudocyst. For the evaluation of cystic or cavitary lung lesion in infants and children, imaging plays an important role in accurate early diagnosis and optimal patient management. Therefore, a practical imaging approach based on the most sensitive and least invasive imaging modality in an efficient and cost-effective manner is paramount. We reviewed the conventional radiographs and computed tomography findings of the most common cystic and cavitary lung lesions in infants and children. PMID:24605255

  14. [Foot lesions].

    PubMed

    Stelzner, C; Schellong, S; Wollina, U; Machetanz, J; Unger, L

    2013-11-01

    The foot is the target organ of a variety of internal diseases. Of upmost importance is the diabetic foot syndrome (DFS). Its complex pathophysiology is driven by the diabetic neuropathy, a vastly worsening effect is contributed by infection and ischemia. Seemingly localised lesions have the potential for phlegmone and septicaemia if not diagnosed and drained early. The acral lesions of peripheral artery occlusive disease (PAOD) have unique features as well. However, their life-threatening potential is lower than that of DFS even if the limb is critical. Notably, isolated foot lesions with a mere venous cause may arise from insufficient perforator veins; the accompanying areas of haemosiderosis will lead the diagnostic path. Cholesterol embolization (blue toe syndrome, trash foot) elicits a unique clinical picture and will become more frequent with increasing numbers of catheter-based procedures. Finally, descriptions are given of podagra and of foot mycosis as disease entities not linked to perfusion. The present review focuses on the depiction of disease and its diagnosis, leaving therapeutic considerations untouched. PMID:24114468

  15. Histiocytosis X: clinical trial of chlorambucil: a report from Childrens Cancer Study Group.

    PubMed

    Lahey, M E; Heyn, R M; Newton, W A; Shore, N; Smith, W B; Leikin, S; Hammond, D

    1979-01-01

    A prospective study for histiocytosis X was designed to determine whether "good risk" patients, ie, those without evidence of dysfunction of liver, lung, or hemopoietic system, would respond to single agent therapy; in this case chlorambucil (CMB) used in a dose of 5 mgm/m2/day. If there was no response after an adequate trial period, treatment was initiated with four drugs using a combination of prednisone, vinblastine, cyclophosphamide and methotrexate. There were 26 evaluable patients, 57% of whom were less than two years of age at onset of therapy. There were three complete and four partial responses to CMB for a response rate of 26.9%. Sixteen patients received an adequate trial of four-drug therapy with three complete and two partial responses for a response rate of 33%. These responses were inferior to those previously reported for either single agents or combined therapy in histiocytosis X. PMID:396466

  16. Smoking and interstitial lung disease. The effect of cigarette smoking on the incidence of pulmonary histiocytosis X and sarcoidosis.

    PubMed

    Hance, A J; Basset, F; Saumon, G; Danel, C; Valeyre, D; Battesti, J P; Chrétien, J; Georges, R

    1986-01-01

    Cigarette smoking produces marked alterations in the lung parenchyma and in the population of immune and inflammatory cells present in the lower respiratory tract. These cigarette-induced changes appear to influence the incidence of two different interstitial lung diseases, histiocytosis X and sarcoidosis. Smoking is a strong risk factor for the development of pulmonary histiocytosis X, since the incidence of smoking is very high among patients with histiocytosis X: 90% of the patients with histiocytosis X were smokers; 46% of the controls were smokers (p less than .001). In contrast, smoking appears to reduce the incidence of sarcoidosis: 31% of the patients with sarcoidosis were smokers (p less than .05 compared to controls). In an effort to understand how cigarette smoking influences the incidence of these two disorders, we compared the numbers and types of immune and inflammatory cells recovered by bronchoalveolar lavage from nonsmoking and smoking controls and patients with histiocytosis X and sarcoidosis. Although nonsmoking patients with histiocytosis X did not have a significant increase in the number of alveolar macrophages recovered by lavage (p greater than .2 compared to normals), smoking patients had an increase in the number of alveolar macrophages similar to that observed in the control population. In contrast, the number of macrophages recovered from patients with sarcoidosis who smoked was considerably less than that observed in normal smokers (p less than .05 comparing patients with sarcoidosis and controls who smoked 1-20 cigarettes/day). This difference in the intensity of the cigarette-induced macrophage alveolitis observed in the two patient groups may be important in explaining the opposite effects of cigarette smoking on the incidence of histiocytosis X and sarcoidosis. PMID:3488004

  17. Laryngeal Langerhans Cell Histiocytosis Presenting with Neck Mass in an Adult Woman

    PubMed Central

    Jahandideh, Hesam; Nasoori, Yasser; Rostami, Sara; Safdarian, Mahdi

    2016-01-01

    Langerhans cell histiocytosis (LCH) is a very rare condition that commonly affects the head and neck region. There are very few cases of isolated laryngeal involvement by LCH, mostly reported in pediatric patients. Here, we report a case of laryngeal LCH in a 62-year-old woman presenting with a neck mass several weeks ago. The clinical and histopathological findings are reported with a brief discussion about the disease. PMID:27127670

  18. Rare case of unifocal Langerhans cell histiocytosis in four-month-old child.

    PubMed

    Martins, Marco Antonio T; Gheno, José Luis N; Sant'Ana Filho, Manoel; Pinto, Décio S; Tenis, Carlos Alberto; Martins, Manoela D

    2011-07-01

    Langerhans cell histiocytosis (LCH) comprises a group of disorders, the common feature of which is Langerhans cell proliferation. The clinical presentation is highly varied. The severity and prognosis of the disease are dependent on the type and extent of organ involvement. This paper reports a rare case of a four-month-old white male with unifocal LCH limited exclusively to the mandible, discussing the diagnosis, radiographic and immunohistochemical aspects, treatment and monitoring multidisciplinary of the case. PMID:21592594

  19. The adverse prognostic hallmarks in identical twins with Langerhans cell histiocytosis: a clinical report and literature review.

    PubMed

    Chai, Damin; Tao, Yisheng; Bao, Zhengqi; Yang, Li; Feng, Zhenzhong; Ma, Li; Liang, Limei; Zhou, Xinwen

    2013-01-01

    Langerhans cell histiocytosis (LCH) is characterized by uncontrolled proliferation of Langerhans cells accompanying eosinophils. It often attacks children under 10 years of age. LCH in identical twins is very rare and its prognosis is different. Here we report identical-twin sisters with LCH. Computed tomography (CT) revealed osteolytic change in each twin's skull, and the elder exhibited poor eyesight. There were massive histiocyte-like cells surrounded by eosinophils in pathologic specimen of the abnormal lesions, which is typical pathologic finding in LCH. These pathologic cells were positive for S-100 and the cell surface protein CD1 antigen (CD1α), the known markers of LCH. After treating them with surgery, no symptoms were seen in the younger until now. While the older was found another soft mass (about 2.0 cm in diameter) in the left temporal area 18 months later. The same treatment was given to the older after admission, and she is healthy to date. To explore the relationship between hallmarks and the prognosis of identical-twin patients with LCH, we retrieved the 16 literatures (16 identical-twin pairs, 31 patients) listed in PubMed during the past 60 years. The data revealed all those patients who have disseminated to the bone marrow, spleen and liver with symptoms of fever and hepatosplenomegaly exhibited worse prognosis (9 out of the 31 patients). The other identical-twin subjects without infiltration of those organs recovered well. In conclusion, this study reveals the adverse hallmarks of prognosis in identical-twin patients with LCH by reviewing relevant literatures. PMID:23924960

  20. Orbit to orbit transportation

    NASA Technical Reports Server (NTRS)

    Bergeron, R. P.

    1980-01-01

    Orbital transfer vehicle propulsion options for SPS include both chemical (COTV) and electrical (EOTV) options. The proposed EOTV construction method is similar to that of the SPS and, by the addition of a transmitting antenna, may serve as a demonstration or precursor satellite option. The results of the studies led to the selection of a single stage COTV for crew and priority cargo transfer. An EOTV concept is favored for cargo transfer because of the more favorable orbital burden factor over chemical systems. The gallium arsenide solar array is favored over the silicon array because of its self annealing characteristics of radiation damage encountered during multiple transitions through the Van Allen radiation belt. Transportation system operations are depicted. A heavy lift launch vehicle (HLLV) delivers cargo and propellants to LEO, which are transferred to a dedicated EOTV by means of an intraorbit transfer vehicle (IOTV) for subsequent transfer to GEO. The space shuttle is used for crew transfer from Earth to LEO. At the LEO base, the crew module is removed from the shuttle cargo bay and mated to a COTV for transfer to GEO. Upon arrival at GEO, the SPS construction cargo is transferred from the EOTV to the SPS construction base by IOTV. Crew consumables and resupply propellants are transported to GEO by the EOTV. Transportation requirements are dominated by the vast quantity of materials to be transported to LEO and GEO.

  1. A Case of Orbital Histoplasmosis.

    PubMed

    Krakauer, Mark; Prendes, Mark Armando; Wilkes, Byron; Lee, Hui Bae Harold; Fraig, Mostafa; Nunery, William R

    2016-01-01

    Histoplasma capsulatum var capsulatum is a dimorphic fungus endemic to the Ohio and Mississippi River Valleys of the United States. In this case report, a 33-year-old woman who presented with a right orbital mass causing progressive vision loss, diplopia, and facial swelling is described. Lateral orbitotomy with lateral orbital wall bone flap was performed for excisional biopsy of the lesion. The 1.5 × 1.8 × 2.3 cm cicatricial mass demonstrated a granulomatous lesion with necrosis and positive staining consistent with Histoplasma capsulatum var capsulatum infection. To the authors' knowledge, this is the first case of orbital histoplasmosis to be reported in the United States and the first case worldwide of orbital histoplasmosis due to Histoplasma capsulatum var capsulatum. PMID:25186215

  2. Orbital cellulitis

    MedlinePlus

    ... Haemophilus influenzae B) vaccine. The bacteria Staphylococcus aureus , Streptococcus pneumoniae , and beta-hemolytic streptococci may also cause orbital cellulitis. Orbital cellulitis infections in children may get worse very quickly and can lead ...

  3. Orbital pseudotumor

    MedlinePlus

    ... Names Idiopathic orbital inflammatory syndrome (IOIS) Images Skull anatomy References Goodlick TA, Kay MD, Glaser JS, Tse DT, Chang WJ. Orbital disease and neuro-ophthalmology. In: Tasman W, Jaeger EA, eds. Duane’s ...

  4. Kepler's Orbit

    NASA Video Gallery

    Kepler does not orbit the Earth, rather it orbits the Sun in concert with the Earth, slowly drifting away from Earth. Every 61 Earth years, Kepler and Earth will pass by each other. Throughout the ...

  5. Combined Cutaneous Rosai-Dorfman Disease and Localized Cutaneous Langerhans Cell Histiocytosis Within a Single Subcutaneous Nodule

    PubMed Central

    Litzner, Brandon R.; Subtil, Antonio

    2015-01-01

    Abstract: Rosai-Dorfman disease (RDD) is a reactive multisystem histiocytosis that typically presents with cervical lymphadenopathy and systemic symptoms. Cutaneous involvement occurs in approximately 10% of cases, and 3% of cases are limited to the skin without nodal or other extranodal involvement. Langerhans cell histiocytosis (LCH) is a clonal histiocytosis with a wide spectrum of presentations ranging from isolated skin or bone disease to multisystem involvement. Rare case reports have identified concomitant presentation of RDD and LCH; however, most of these reports have involved LCH and RDD occurring concurrently but at separate sites. We present a rare case of concurrent RDD and LCH presenting within a single skin nodule. The patient did not have any evidence of systemic involvement and has remained stable without additional treatment. We also review the literature on this unusual co-presentation and suggest possible underlying mechanisms. Finally, we recommend baseline laboratory and imaging studies and discuss treatment options based on the available evidence. PMID:26588339

  6. Extra Nodal Rosai-Dorfman Disease (Sinus Histiocytosis with Massive Lymphadenopathy) Presenting as Asymmetric Bilateral Optic Atrophy : An Atypical Ocular Presentation.

    PubMed

    Shukla, Eesha; Nicholson, Anjali; Agrawal, Anamika; Rathod, Darshana

    2016-09-01

    Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy, SHML) is a rare, non-hereditary, benign histiocytic proliferative disorder, presenting as painless bilateral cervical lymphadenopathy, with systemic symptoms. Extra nodal manifestations have been reported in 28-43 % cases with rare ocular involvement. We report a case of a 57 year old female presenting with gradual progressive decrease of vision OU since 8 months associated with epistaxis. Fundus examination revealed established optic atrophy in right eye with features of chronic papilloedema in left eye suggestive of compressive lesion. CT of brain, paranasal sinuses confirmed the presence of homogenously enhancing mass in left ethmoid sinus, left sphenoid sinus extending into suprasellar region. The biopsy of this mass revealed extra nodal SHML with tissue sections being S100 and CD68 positive with emperipolesis noted. Here we describe this atypical ocular presentation of extra nodal SHML to highlight that this rare disease can manifest as an aggressive sight threatening entity, even in older age group. PMID:27091209

  7. 18F-fluorodeoxyglucose positron emission tomography/computed tomography for primary thyroid langerhans histiocytosis: A case report and literature review.

    PubMed

    Long, Qi; Shaoyan, Wang; Hui, Wang

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare clonal proliferative disease, with an incidence rate of 4.0-5.4/1 million individuals. LCH encompasses a spectrum of disorders with diverse clinical presentations ranging from a single organ to multiple organ involvement. LCH rarely involves the thyroid gland. We presented a case with LCH of thyroid gland. The patient had painless progressive neck enlargement and then diabetes insipidus. Ultrasonic scan and magnetic resonance imaging scan revealed nodular goiter and pituitary stalk enlargement, respectively. Histopathological analysis revealed features of histiocytoid cells. 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) was performed in order to rule out the presence of whole body infiltration. 18F-FDG PET/CT also demonstrated increased uptake in the thickening pituitary stalk and maxillofacial skin lesion, in addition to the bilateral thyroid nodules, CT showed the left lung nodule and the skull destruction without 18F-FDG uptake. This report emphasizes the role of 18F-FDG PET/CT in multiple organs involvement of patients with LCH. PMID:26430317

  8. 18F-fluorodeoxyglucose positron emission tomography/computed tomography for primary thyroid langerhans histiocytosis: A case report and literature review

    PubMed Central

    Long, Qi; Shaoyan, Wang; Hui, Wang

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare clonal proliferative disease, with an incidence rate of 4.0–5.4/1 million individuals. LCH encompasses a spectrum of disorders with diverse clinical presentations ranging from a single organ to multiple organ involvement. LCH rarely involves the thyroid gland. We presented a case with LCH of thyroid gland. The patient had painless progressive neck enlargement and then diabetes insipidus. Ultrasonic scan and magnetic resonance imaging scan revealed nodular goiter and pituitary stalk enlargement, respectively. Histopathological analysis revealed features of histiocytoid cells. 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) was performed in order to rule out the presence of whole body infiltration. 18F-FDG PET/CT also demonstrated increased uptake in the thickening pituitary stalk and maxillofacial skin lesion, in addition to the bilateral thyroid nodules, CT showed the left lung nodule and the skull destruction without 18F-FDG uptake. This report emphasizes the role of 18F-FDG PET/CT in multiple organs involvement of patients with LCH. PMID:26430317

  9. S100-Negative, CD1a-Positive Cutaneous Histiocytosis in a Patient with S100-Positive, CD1a-Positive Pulmonary Histiocytosis.

    PubMed

    Mask-Bull, Lisa; Crowson, Neil A; John, Andrew; Mask, Neal A

    2015-08-01

    In the diagnostic approach to histiocytic proliferations, immunohistochemistry may be a source of both confusion and clarification. We present a case of a 60-year-old man with a generalized pruritic eruption that demonstrated positive staining for CD1a, but negative staining for langerin and S100 protein. This immunophenotype is neither representative nor characteristic of any recognized dendritic cell tumor but has been previously described in 3 cases of skin-limited histiocytosis. However, our patient also demonstrated pulmonary histiocytic infiltrates that were positive for both CD1a and S100 proteins. This differing expression of S100 protein witnessed in 2 separate organ systems affords us insight into the pathophysiology of these histiocytic proliferations. PMID:25321083

  10. [Orbital inflammation].

    PubMed

    Mouriaux, F; Coffin-Pichonnet, S; Robert, P-Y; Abad, S; Martin-Silva, N

    2014-12-01

    Orbital inflammation is a generic term encompassing inflammatory pathologies affecting all structures within the orbit : anterior (involvement up to the posterior aspect of the globe), diffuse (involvement of intra- and/or extraconal fat), apical (involvement of the posterior orbit), myositis (involvement of only the extraocular muscles), dacryoadenitis (involvement of the lacrimal gland). We distinguish between specific inflammation and non-specific inflammation, commonly referred to as idiopathic inflammation. Specific orbital inflammation corresponds to a secondary localization of a "generalized" disease (systemic or auto-immune). Idiopathic orbital inflammation corresponds to uniquely orbital inflammation without generalized disease, and thus an unknown etiology. At the top of the differential diagnosis for specific or idiopathic orbital inflammation are malignant tumors, represented most commonly in the adult by lympho-proliferative syndromes and metastases. Treatment of specific orbital inflammation begins with treatment of the underlying disease. For idiopathic orbital inflammation, treatment (most often corticosteroids) is indicated above all in cases of visual loss due to optic neuropathy, in the presence of pain or oculomotor palsy. PMID:25455557

  11. Lactate dehydrogenase isoenzyme patterns in blood cells from histiocytosis X children.

    PubMed

    Perlino, E; Marra, E; Maenza, S; de Terlizzi, M; Coppola, B C; Santostasi, T; Quagliariello, E

    1993-12-31

    To find a clinical assay for histiocytosis X (HX) diagnosis, measurements were made of both activity and isoenzyme distribution of lactate dehydrogenase (LDH; EC 1.1.1.27) from the blood cells of 6 acute phase and 9 remission patients. A significant increase in the LDH activity measured in the monocytes and lymphocytes isolated from the blood of the acute phase patients was found. The increased activity was due to an enhancement of the normal pattern of LDH isoenzymes in these cells and not to a change in isoenzyme distribution. No increase was found in monocyte LDH isoenzymes from the patients in remission. PMID:8143371

  12. Langerhans cell histiocytosis of the cervical spine in an adult: a case report.

    PubMed

    Sayhan, Salih; Altinel, Deniz; Erguden, Cenk; Kizmazoglu, Ceren; Guray, Merih; Acar, Umit

    2010-07-01

    We present a case of a 47-year-old-woman with a complaint of cervical pain with paresthetic appearance on her left arm. She was treated with analgetics. Further radiological evaluation because of the persistent pain revealed an osteolytic destruction of the fourth cervical vertebra. The patient underwent anterior cervical corpectomy with total excision of the tumor. Stabilization of the cervical spine was performed. Histology confirmed the diagnosis of Langerhans cell histiocytosis (LCH) of the cervical spine. This case report presents the histopathological evaluation, diagnostic work-up and the treatment procedures because of rarity of cervical spinal LCH cases in the literature. PMID:20669118

  13. [Symptomatic Langerhans-cell-histiocytosis of the cervical spine in a child: case report].

    PubMed

    Schroers, C; Donauer, E; Laudan, M; Herbst, E W; Barz, H

    2000-01-01

    We report on a six year old female presented with a painful torticollis and a hemidysaesthesia caused by destruction of the third cervical vertebra and a paravertebral soft-tissue mass. At diagnostic routine finally a biopsy gives the diagnosis of Langerhans cell histiocytosis. In a second open approach the destructed vertebral body was replaced by a precisely adjusted autologous bone interponate and the patient was maintained in halo vest immobilisation. The outcome is described and an overview of the current literature is given. PMID:10916784

  14. Pulmonary Langerhans Cell Histiocytosis-associated Pulmonary Hypertension Showing a Drastic Improvement Following Smoking Cessation.

    PubMed

    Kinoshita, Yoshiaki; Watanabe, Kentaro; Sakamoto, Atsuhiko; Hidaka, Kouko

    2016-01-01

    Pulmonary Langerhans cell histiocytosis (PLCH) is a rare, smoking-related, interstitial lung disease, and pulmonary hypertension (PH) is associated with mortality. We herein report a case of PLCH complicated by severe PH and respiratory impairment. After developing PH, the patient displayed a cystic pattern on chest high-resolution computed tomography (HRCT). This, in turn, corresponded with the scarring stage of PLCH. However, the patient's PH and respiratory impairment improve dramatically following smoking cessation. PLCH patients with a cystic pattern on chest HRCT may still be able to improve their PH and respiratory impairment when they are able to quit smoking. PMID:26935369

  15. Myths in the Diagnosis and Management of Orbital Tumors.

    PubMed

    Gündüz, Kaan; Yanık, Özge

    2015-01-01

    Orbital tumors constitute a group of diverse lesions with a low incidence in the population. Tumors affecting the eye and ocular adnexa may also secondarily invade the orbit. Lack of accumulation of a sufficient number of cases with a specific diagnosis at various orbital centers, the paucity of prospective randomized studies, animal model studies, tissue bank, and genetic studies led to the development of various myths regarding the diagnosis and treatment of orbital lesions in the past. These myths continue to influence the diagnosis and treatment of orbital lesions by orbital specialists. This manuscript discusses some of the more common myths through case summaries and a review of the literature. Detailed genotypic analysis and genetic classification will provide further insight into the pathogenesis of many orbital diseases in the future. This will enable targeted treatments even for diseases with the same histopathologic diagnosis. Phenotypic variability within the same disease will be addressed using targeted treatments. PMID:26692710

  16. Myths in the Diagnosis and Management of Orbital Tumors

    PubMed Central

    Gündüz, Kaan; Yanık, Özge

    2015-01-01

    Orbital tumors constitute a group of diverse lesions with a low incidence in the population. Tumors affecting the eye and ocular adnexa may also secondarily invade the orbit. Lack of accumulation of a sufficient number of cases with a specific diagnosis at various orbital centers, the paucity of prospective randomized studies, animal model studies, tissue bank, and genetic studies led to the development of various myths regarding the diagnosis and treatment of orbital lesions in the past. These myths continue to influence the diagnosis and treatment of orbital lesions by orbital specialists. This manuscript discusses some of the more common myths through case summaries and a review of the literature. Detailed genotypic analysis and genetic classification will provide further insight into the pathogenesis of many orbital diseases in the future. This will enable targeted treatments even for diseases with the same histopathologic diagnosis. Phenotypic variability within the same disease will be addressed using targeted treatments. PMID:26692710

  17. Orbiter's Skeleton

    NASA Technical Reports Server (NTRS)

    2005-01-01

    The structure of NASA's Mars Reconnaissance Orbiter spacecraft is constructed from composite panels of carbon layers over aluminum honeycomb, lightweight yet strong. This forms a basic structure or skeleton on which the instruments, electronics, propulsion and power systems can be mounted. The propellant tank is contained in the center of the orbiter's structure. This photo was taken at Lockheed Martin Space Systems, Denver, during construction of the spacecraft.

  18. Atherectomy in complex infrainguinal lesions: a review.

    PubMed

    Engelberger, S; van den Berg, J C

    2015-02-01

    In the femoropopliteal segment, endovascular revascularization techniques have gained the role as a first line treatment strategy. Nitinol stent placement has improved the short- and mid-term primary patency rates in most lesion types and is therefore widely applied. Stenting has several shortcomings as in-stent restenosis, stent fractures and foreign material being left behind in the vessel. The concept of atherectomy is plaque debulking. This results in a potential reduction of inflation pressure requirements in angioplasty. Stent placement and consecutive in-stent restenosis may be avoided. In this non systematic literature review, the performance of different atherectomy techniques, such as direct atherectomy, orbital atherectomy, laser debulking and rotational atherectomy in the treatment of complex femoropopliteal lesions, including long lesions, moderately to heavily calcified lesions as well as occlusions and in-stent restenosis, has been analyzed. PMID:25399550

  19. Langerhans Cell Histiocytosis in an Infant Mimicking a Lymphoma at Presentation

    PubMed Central

    Madasu, Anjan; Noor Rana, Asim; Banat, Saleh; Humad, Hani; Mustafa, Rashid; AlJassmi, Abdulrahman Mohd

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare disorder characterized by proliferation and accumulation of clonal dendritic cells with varied clinical presentation and an unpredictable course. We report a 5-month-old infant with LCH who presented with severe respiratory distress, a large mediastinal mass, significant generalized lymphadenopathy, and hepatosplenomegaly. Lymphoma, especially T cell lymphoblastic lymphoma, can present with superior mediastinal syndrome needing urgent empirical therapy without biopsy. However, lack of response prompted a biopsy which confirmed it to be a case of LCH and that leads to appropriate therapy and survival. There have been reports of LCH presenting with isolated mediastinal mass or with generalized lymphadenopathy, but the combined presentation of generalized lymphadenopathy with large mediastinal mass, hepatosplenomegaly, and fever in an infant has rarely been reported. Conclusion. LCH should also be considered in the differential diagnosis of an infant presenting with generalized lymphadenopathy, mediastinal mass, hepatosplenomegaly, and fever. PMID:26587301

  20. Pulmonary Langerhans Cell Histiocytosis and Diabetes Insipidus in a Young Smoker

    PubMed Central

    Earlam, K.; Souza, C. A.; Glikstein, R.; Gomes, M. M.; Pakhalé, S.

    2016-01-01

    Langerhans cell histiocytosis is characterized by the abnormal nodular proliferation of histiocytes in various organ systems. Pulmonary involvement seen in young adults is nearly always seen in the context of past or current cigarette smoking. Although it tends to be a single-system disease, extrapulmonary manifestations involving the skin, bone, and hypothalamic-pituitary-axis are possible. High resolution CT (HRCT) of the thorax findings includes centrilobular nodules and cysts that are bizarre in shape, variable in size, and thin-walled. Often the diagnosis can be made based on the appropriate clinical presentation and typical imaging findings. Treatment includes smoking cessation and the potential use of glucocorticoids or cytotoxic agents depending on the severity of disease and multisystem involvement.

  1. CNS involvement of Langerhans cell histiocytosis. Report of 23 surgically treated cases.

    PubMed

    Belen, D; Colak, A; Ozcan, O E

    1996-01-01

    We present 23 cases of Langerhans cell histiocytosis with central nervous system (CNS) involvement. The major complaints were a mass on the cranial vault in fifteen (65%), visual disturbance in four (16%), polyurea-polydipsia in three (13%), and progressive weakness in all extremities in one patient (4%). Neurological examination revealed no abnormality in sixteen patients (70%), cranial nerve palsy, visual field defect and optic atrophy in six (26%) and paraparesis in one (4%). Tumoral mass was found to be located on the cranial vault (65%), in the suprasellar region (21%) and in the spinal column (8%). The cranium and spinal column were both involved in one patient. All patients underwent surgery; craniectomy with grossly total tumor excision plus cranioplasty (65%), craniotomy with subtotal tumor excision (26%), and vertebrectomy with grafting (13%) were performed. The clinical, radiological and histopathological features, as well as therapeutical considerations are discussed and the pertinent literature is reviewed. PMID:9007888

  2. Langerhans cell histiocytosis of the cervical spine: a single institution experience in four patients.

    PubMed

    Tan, Grace; Samson, Ignace; De Wever, Ivo; Goffin, Jan; Demaerel, Philippe; Van Gool, Stefaan W

    2004-03-01

    When Langerhans cell histiocytosis (LCH) occurs at critical sites, such as in the cervical spine, there is a substantial risk for morbidity. Therefore, reports on clinical experiences with those patients remain important. We summarize the history of four patients with unifocal LCH at the cervical spine. All four patients received a biopsy to prove the histopathological diagnosis of LCH by demonstration of CD1a+cells. They were treated with oral prednisolone. All patients recovered completely and kept a normal function of the cervical spine. No reactivation of the disease occurred with an observation time of 3.4-7.3 years. This report contributes to the clinical experience for the treatment of LCH at critical sites. PMID:15076592

  3. Rare interstitial lung disease: Pulmonary Langerhans Cell Histiocytosis in a young non smoking Indian female.

    PubMed

    Fernandes, Lalita; Vadala, Rohit; Mesquita, Anthony Menezes; Vaideeswar, Pradeep

    2015-01-01

    Adult Pulmonary Langerhans Cell Histiocytosis (PLCH) is a rare interstitial lung disease which occurs almost exclusively in smokers. A marked male predominance was initially reported, but recent studies show both men and women are equally affected due to the increasing smoking habits in women. The natural history is variable with 25% of patients having asymptomatic disease while 10-20% progress rapidly to respiratory insufficiency and death. The diagnosis is not easily recognized by clinicians or pathologists. Awareness of the clinical presentation and classical HRCT findings helps in early diagnosis and management of this disease. We report a rare case of severe PLCH in a young non smoking female with a short history who progressed rapidly to respiratory failure and died. PMID:25857566

  4. Dramatic and sustained responsiveness of pulmonary Langerhans cell histiocytosis-associated pulmonary hypertension to vasodilator therapy.

    PubMed

    May, Adam; Kane, Garvan; Yi, Eunhee; Frantz, Robert; Vassallo, Robert

    2015-01-01

    Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon diffuse lung disease characterized by the abnormal accumulation of Langerhans' cells around small airways and other distal lung compartments. Although pulmonary hypertension (PH) is a frequent complication of PLCH, the role of advanced PH therapies for PLCH-related PH is not well-established. We describe a PLCH patient with severe, disease-related PH that responded unexpectedly well to advanced PH therapy with sustained improvement over a 10 year follow-up period. This case indicates that PLCH-associated PH may, in certain instances, be highly responsive to advanced PH therapies and emphasizes the importance of trialing these therapies among patients with PLCH-related PH. PMID:26029568

  5. A case of pulmonary Langerhans cell histiocytosis in a young woman with coeliac disease.

    PubMed

    Mastruzzo, Claudio; Vancheri, Carlo; Li Mura, Dario; Poletti, Venerino; Failla, Marco; Crimi, Nunzio

    2007-06-01

    Pulmonary Langerhans cells histiocytosis (pulmonary LCH) is an idiopathic unusual lung disease and its association with other systemic diseases has been rarely observed. Here, we describe a young non-smoking woman with concomitant pulmonary LCH and coeliac disease that, despite therapy, suddenly deteriorated. To the best of the authors' knowledge, this is the first report in the medical literature describing an association of coeliac disease with pulmonary LCH. Considering the concomitant occurrence of both diseases in our patient and the severe course of pulmonary LCH observed, we hypothesise that coeliac disease and pulmonary LCH might be related by a common disturbance in immunity and the onset and/or the course of pulmonary LCH could be influenced or markedly worsened by the presence of coeliac disease. PMID:17629812

  6. Dramatic and sustained responsiveness of pulmonary Langerhans cell histiocytosis-associated pulmonary hypertension to vasodilator therapy

    PubMed Central

    May, Adam; Kane, Garvan; Yi, Eunhee; Frantz, Robert; Vassallo, Robert

    2014-01-01

    Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon diffuse lung disease characterized by the abnormal accumulation of Langerhans' cells around small airways and other distal lung compartments. Although pulmonary hypertension (PH) is a frequent complication of PLCH, the role of advanced PH therapies for PLCH-related PH is not well-established. We describe a PLCH patient with severe, disease-related PH that responded unexpectedly well to advanced PH therapy with sustained improvement over a 10 year follow-up period. This case indicates that PLCH-associated PH may, in certain instances, be highly responsive to advanced PH therapies and emphasizes the importance of trialing these therapies among patients with PLCH-related PH. PMID:26029568

  7. Orbital Debris

    NASA Technical Reports Server (NTRS)

    Kessler, D. J. (Compiler); Su, S. Y. (Compiler)

    1985-01-01

    Earth orbital debris issues and recommended future activities are discussed. The workshop addressed the areas of environment definition, hazards to spacecraft, and space object management. It concluded that orbital debris is a potential problem for future space operations. However, before recommending any major efforts to control the environment, more data are required. The most significant required data are on the population of debris smaller than 4 cm in diameter. New damage criteria are also required. When these data are obtained, they can be combined with hypervelocity data to evaluate the hazards to future spacecraft. After these hazards are understood, then techniques to control the environment can be evaluated.

  8. Orbital cellulitis.

    PubMed Central

    Martin-Hirsch, D P; Habashi, S; Hinton, A H; Kotecha, B

    1992-01-01

    Orbital cellulitis is an emergency. It may cause blindness and progress to life-threatening sequelae such as brain abscess, meningitis and cavernous sinus thrombosis. Successful management is dependent upon urgent referral and immediate treatment. Although isolated eyelid erythema and swelling usually indicate primary infection anterior to the orbital septum, they may also be the first signs of an underlying frontal or ethmoidal sinusitis. The condition always requires emergency referral to both an ophthalmologist and otorhinolaryngologist. Images Fig. 1 Fig. 2 Fig. 3 Fig. 4 PMID:1388488

  9. Example based lesion segmentation

    NASA Astrophysics Data System (ADS)

    Roy, Snehashis; He, Qing; Carass, Aaron; Jog, Amod; Cuzzocreo, Jennifer L.; Reich, Daniel S.; Prince, Jerry; Pham, Dzung

    2014-03-01

    Automatic and accurate detection of white matter lesions is a significant step toward understanding the progression of many diseases, like Alzheimer's disease or multiple sclerosis. Multi-modal MR images are often used to segment T2 white matter lesions that can represent regions of demyelination or ischemia. Some automated lesion segmentation methods describe the lesion intensities using generative models, and then classify the lesions with some combination of heuristics and cost minimization. In contrast, we propose a patch-based method, in which lesions are found using examples from an atlas containing multi-modal MR images and corresponding manual delineations of lesions. Patches from subject MR images are matched to patches from the atlas and lesion memberships are found based on patch similarity weights. We experiment on 43 subjects with MS, whose scans show various levels of lesion-load. We demonstrate significant improvement in Dice coefficient and total lesion volume compared to a state of the art model-based lesion segmentation method, indicating more accurate delineation of lesions.

  10. Immature sinus histiocytosis. Light- and electron-microscopic features, immunologic phenotype, and relationship with marginal zone lymphocytes.

    PubMed Central

    van den Oord, J. J.; de Wolf-Peeters, C.; De Vos, R.; Desmet, V. J.

    1985-01-01

    The light-microscopic, ultrastructural, and immunohistochemical features of immature sinus histiocytosis were studied in 10 lymph nodes with the histologic picture of toxoplasmic lymphadenitis and compared with the features of lymphoid cells present in the marginal zone of the splenic white pulp. Areas of immature sinus histiocytosis consisted largely of medium-sized lymphoid cells with markedly irregular nuclei and abundant pale cytoplasm. Using a panel of monoclonal antibodies, the predominating lymphoid cells were found to carry the B-cell phenotype B1+Ba1-sIgM+sIgD-OKIa1+. Admixed were variable numbers of larger, blastic lymphoid cells, small lymphocytes, histiocytic elements, and polymorphonuclear granulocytes. The marginal zone of the splenic white pulp was composed of a similar mixture of cells, and marginal-zone lymphocytes demonstrated an analogous immunohistochemical phenotype. Our results indicate that immature sinus histiocytes are B-lymphoid cells that are closely related to marginal zone lymphocytes. As such, immature sinus histiocytes may have a role similar to that of marginal-zone lymphocytes, which have been claimed to transport antigens or immune complexes toward the follicular center or to serve as precursors of plasma cells. We suggest that immature sinus histiocytosis represents an abnormal expansion of the marginal zone, normally present at the sinusoidal pole of lymphoid follicles. The reason for this marginal-zone hyperplasia, recognized as immature sinus histiocytosis in a variety of reactive lymph node conditions, may be a maturation arrest in the normal development of immature sinus histiocytes into small, sIgM+ sIgD+ lymphocytes. Images Figure 3 Figure 1 Figure 2 Figure 4 Figure 5 Figure 6 Figure 7 Figure 8 Figure 9 Figure 10 Figure 11 Figure 12 Figure 13 Figure 14 PMID:3970140

  11. Diagnosis of Langerhans Cell Histiocytosis on Fine Needle Aspiration Cytology: A Case Report and Review of the Cytology Literature

    PubMed Central

    Kumar, Neeta; Sayed, Shahin; Vinayak, Sudhir

    2011-01-01

    A case of multifocal Langerhans cell histiocytosis in a two-year-old child is presented where fine needle aspiration was helpful in achieving a rapid and accurate diagnosis in an appropriate clinical and radiological setting. This can avoid unnecessary biopsy and guide the management especially where access to histopathology is limited. The highly characteristic common and rare cytological features are highlighted with focus on differential diagnoses and causes of pitfalls. PMID:21331166

  12. Solitary fibrous tumor of the orbit.

    PubMed

    Gupta, Sumiti; Verma, Renuka; Sen, Rajeev; Singh, Ishwar; Marwah, Nisha; Kohli, Rachneet

    2016-01-01

    Solitary fibrous tumor (SFT) is a rare spindle-cell neoplasm originating from the mesenchyme. It was originally thought to occur exclusively in the intrathoracic region but has been recently described in extrapleural sites including the orbit. SFT of the orbit is a rare lesion, which can be misdiagnosed as hemangiopericytoma, fibrous histiocytoma, meningioma, or neurofibroma. Immunohistochemistry plays an important role. We report an orbital SFT in a 39-year-old female presented with painless, progressive proptosis, and diminished vision in the right eye for the duration of 6 months. Magnetic resonance imaging demonstrated well-defined enhancing mass lesion. The patient underwent complete tumor removal through a right fronto-orbital approach, and a pathological diagnosis of the solitary fibrous tumor was made. Postoperatively, the patient was symptom-free. Clinical and pathological findings including immunohistochemistry are presented along with a brief discussion of literature. PMID:26889300

  13. Solitary fibrous tumor of the orbit

    PubMed Central

    Gupta, Sumiti; Verma, Renuka; Sen, Rajeev; Singh, Ishwar; Marwah, Nisha; Kohli, Rachneet

    2016-01-01

    Solitary fibrous tumor (SFT) is a rare spindle-cell neoplasm originating from the mesenchyme. It was originally thought to occur exclusively in the intrathoracic region but has been recently described in extrapleural sites including the orbit. SFT of the orbit is a rare lesion, which can be misdiagnosed as hemangiopericytoma, fibrous histiocytoma, meningioma, or neurofibroma. Immunohistochemistry plays an important role. We report an orbital SFT in a 39-year-old female presented with painless, progressive proptosis, and diminished vision in the right eye for the duration of 6 months. Magnetic resonance imaging demonstrated well-defined enhancing mass lesion. The patient underwent complete tumor removal through a right fronto-orbital approach, and a pathological diagnosis of the solitary fibrous tumor was made. Postoperatively, the patient was symptom-free. Clinical and pathological findings including immunohistochemistry are presented along with a brief discussion of literature. PMID:26889300

  14. Secondary mucoepidermoid carcinoma of the orbit

    PubMed Central

    Siuw, Chin Pei; Tan, Siow W; Abdul Wahid, Adrena B; Vasudevan, Suresh

    2016-01-01

    A 40-year-old man presented with right eye axial proptosis and ophthalmoplegia for 3 months. Imaging study showed a right intraconal mass with the erosion of the orbital floor. Incisional biopsy revealed mucoepidermoid carcinoma. Nasal endoscopy was normal and systemic tumor screening was negative for a primary source. The patient underwent right orbital exenteration, uncinectomy, nasal and maxillary mucosal biopsy. Malignant cells were found present in the mucosa of maxillary sinus roof and uncinate bone. The postoperative positron emission tomography scan showed residual active lesion in right orbital apex and maxilla but no primary lesion elsewhere. The patient subsequently underwent 35 cycles of postoperative radiotherapy. Primary mucoepidermoid carcinoma of the orbit is rare and typically arises from the lacrimal gland or sac. Those tumors not arising from lacrimal apparatus should be presumed metastatic in origin, and the thorough systemic survey should be undertaken in the search for the primary tumor. PMID:27146939

  15. Secondary mucoepidermoid carcinoma of the orbit.

    PubMed

    Siuw, Chin Pei; Tan, Siow W; Abdul Wahid, Adrena B; Vasudevan, Suresh

    2016-03-01

    A 40-year-old man presented with right eye axial proptosis and ophthalmoplegia for 3 months. Imaging study showed a right intraconal mass with the erosion of the orbital floor. Incisional biopsy revealed mucoepidermoid carcinoma. Nasal endoscopy was normal and systemic tumor screening was negative for a primary source. The patient underwent right orbital exenteration, uncinectomy, nasal and maxillary mucosal biopsy. Malignant cells were found present in the mucosa of maxillary sinus roof and uncinate bone. The postoperative positron emission tomography scan showed residual active lesion in right orbital apex and maxilla but no primary lesion elsewhere. The patient subsequently underwent 35 cycles of postoperative radiotherapy. Primary mucoepidermoid carcinoma of the orbit is rare and typically arises from the lacrimal gland or sac. Those tumors not arising from lacrimal apparatus should be presumed metastatic in origin, and the thorough systemic survey should be undertaken in the search for the primary tumor. PMID:27146939

  16. Acute orbital pseudotumors: classification and CT features

    SciTech Connect

    Nugent, R.A.; Rootman, J.; Robertson, W.D.; Lapointe, J.S.; Harrison, P.B.

    1981-11-01

    Acute orbital pseudotumors are inflammatory lesions of unknown etiology that may affect part or, less often, all of the tissue within the orbit. A retrospective computed tomographic (CT) study of 16 patients demonstrated that these lesions occur in one of five specific anatomic patterns: anterior, posterior, diffuse, lacrimal, or myositic. The most common location was lacrimal followed by anterior psuedotumors. Posterior, diffuse, and myositic pseudotumors were equally frequent. Localization on the basis of clinical features correlated with the CT localization. Illustrative cases of each of the five types are included. The role of CT in evaluating the therapeutic response is discussed.

  17. Eye and orbit ultrasound

    MedlinePlus

    Echography - eye orbit; Ultrasound - eye orbit; Ocular ultrasonography; Orbital ultrasonography ... ophthalmology department of a hospital or clinic. Your eye is numbed with medicine (anesthetic drops). The ultrasound ...

  18. [Surprising white lesions].

    PubMed

    Nolte, J W; van der Waal, I

    2011-09-01

    A 46-year-old man appeared with white lesions of the oral cavity. A previously taken biopsy revealed no classifying diagnosis and treatment with mouth rinse produced no improvement. A new biopsy was taken, on which the pathologist performed additional tests. This resulted in the diagnosis 'syphilis'. The patient was treated with benzylpenicillin and the oral white lesions disappeared. Although nowadays syphilis is rare, special attention is required when noticing these kinds of lesions of the oral cavity. PMID:21957637

  19. Ghost cell lesions

    PubMed Central

    Rajesh, E.; Jimson, Sudha; Masthan, K. M. K.; Balachander, N.

    2015-01-01

    Ghost cells have been a controversy for a long time. Ghost cell is a swollen/enlarged epithelial cell with eosnophilic cytoplasm, but without a nucleus. In routine H and E staining these cells give a shadowy appearance. Hence these cells are also called as shadow cells or translucent cells. The appearance of these cells varies from lesion to lesion involving odontogenic and nonodontogenic lesions. This article review about the origin, nature and significance of ghost cells in different neoplasms. PMID:26015694

  20. Orbit analysis

    SciTech Connect

    Michelotti, L.

    1995-01-01

    The past fifteen years have witnessed a remarkable development of methods for analyzing single particle orbit dynamics in accelerators. Unlike their more classic counterparts, which act upon differential equations, these methods proceed by manipulating Poincare maps directly. This attribute makes them well matched for studying accelerators whose physics is most naturally modelled in terms of maps, an observation that has been championed most vigorously by Forest. In the following sections the author sketchs a little background, explains some of the physics underlying these techniques, and discusses the best computing strategy for implementing them in conjunction with modeling accelerators.

  1. Sinus histiocytosis with massive lymphadenopathy Rosai-Dorfman disease: a unique case presentation.

    PubMed

    Kaltman, Jordan M; Best, Steven P; McClure, Shawn A

    2011-12-01

    Rosai and Dorfman first described sinus histiocytosis with massive lymphadenopathy (SHML) in 1969 with an article detailing 4 cases in which they differentiated this disease entity from the grouping of diseases categorized as histiocytosis X, where it was previously classified. Also known as Rosai-Dorfman disease (RDD), it is clinically characterized as massive, painless, bilateral, symmetric cervical lymphadenopathy, with fever and leukocytosis. An 11-year-old African American boy was referred to our clinic for extraction of a severely decayed tooth #30 and evaluation of a large right-sided neck mass. Initially, the patient had been seen by his general dentist who had diagnosed the mass as an odontogenic abscess. After 2 courses of different antibiotics, no changes in the mass were noted. Subsequently, the patient was sent to the emergency department where CT revealed multiple right-sided neck masses with the largest measuring 4 × 2 cm. The patient underwent an incisional biopsy by otolaryngology and a diagnosis of necrotic lymph tissue was made. Upon our examination, the carious tooth #30 was felt to be an incidental finding and fine-needle aspiration cytology of the largest mass was performed in 2 places. This also provided a diagnosis of necrotic lymph tissue. In concert with the patient and his mother, the decision was made to excise the mass because of psychosocial concerns. A massive right-sided lymph node attached to the submandibular gland was found and excised without complication. Histologic examination with S-100 stain confirmed a diagnosis of RDD. The patient healed well following surgery and has experienced no further lymphadenopathy. This case presentation and review of the literature is unique, as the patient presented with unilateral cervical lymphadenopathy only. Open biopsy and 2 fine-needle aspirations all returned as necrotic lymph tissue. Obtaining the correct diagnosis was additionally hampered by coincidental dental pathology on the

  2. Noninfectious penile lesions.

    PubMed

    Teichman, Joel M H; Sea, Jason; Thompson, Ian M; Elston, Dirk M

    2010-01-15

    Family physicians commonly diagnose and manage penile cutaneous lesions. Noninfectious lesions may be classified as inflammatory and papulosquamous (e.g., psoriasis, lichen sclerosus, angiokeratomas, lichen nitidus, lichen planus), or as neoplastic (e.g., carcinoma in situ, invasive squamous cell carcinoma). The clinical presentation and appearance of the lesions guide the diagnosis. Psoriasis presents as red or salmon-colored plaques with overlying scales, often with systemic lesions. Lichen sclerosus presents as a phimotic, hypopigmented prepuce or glans penis with a cellophane-like texture. Angiokeratomas are typically asymptomatic, well-circumscribed, red or blue papules, whereas lichen nitidus usually produces asymptomatic pinhead-sized, hypopigmented papules. The lesions of lichen planus are pruritic, violaceous, polygonal papules that are typically systemic. Carcinoma in situ should be suspected if the patient has velvety red or keratotic plaques of the glans penis or prepuce, whereas invasive squamous cell carcinoma presents as a painless lump, ulcer, or fungating irregular mass. Some benign lesions, such as psoriasis and lichen planus, can mimic carcinoma in situ or squamous cell carcinoma. Biopsy is indicated if the diagnosis is in doubt or neoplasm cannot be excluded. The management of benign penile lesions usually involves observation or topical corticosteroids; however, neoplastic lesions generally require surgery. PMID:20082512

  3. Preinvasive lesions

    Cancer.gov

    This definition is for allocation of lesions with preinvasive/borderline properties. It is currently aimed at newly identified neoplasms, which may be similar to those described in humans. In mouse pathology, many adenomas may be preinvasive/borderline lesions. However, their inclusion in the preinvasive category can be justified only upon development of better diagnostic criteria.

  4. Imaging Pediatric Vascular Lesions.

    PubMed

    Nguyen, Tuyet A; Krakowski, Andrew C; Naheedy, John H; Kruk, Peter G; Friedlander, Sheila Fallon

    2015-12-01

    Vascular anomalies are commonly encountered in pediatric and dermatology practices. Most of these lesions are benign and easy to diagnose based on history and clinical exam alone. However, in some cases the diagnosis may not be clear. This may be of particular concern given that vascular anomalies may occasionally be associated with an underlying syndrome, congenital disease, or serious, life-threatening condition. Defining the type of vascular lesion early and correctly is particularly important to determine the optimal approach to management and treatment of each patient. The care of pediatric patients often requires collaboration from a multitude of specialties including pediatrics, dermatology, plastic surgery, radiology, ophthalmology, and neurology. Although early characterization of vascular lesions is important, consensus guidelines regarding the evaluation and imaging of vascular anomalies does not exist to date. Here, the authors provide an overview of pediatric vascular lesions, current classification systems for characterizing these lesions, the various imaging modalities available, and recommendations for appropriate imaging evaluation. PMID:26705446

  5. Imaging Pediatric Vascular Lesions

    PubMed Central

    Nguyen, Tuyet A.; Krakowski, Andrew C.; Naheedy, John H.; Kruk, Peter G.

    2015-01-01

    Vascular anomalies are commonly encountered in pediatric and dermatology practices. Most of these lesions are benign and easy to diagnose based on history and clinical exam alone. However, in some cases the diagnosis may not be clear. This may be of particular concern given that vascular anomalies may occasionally be associated with an underlying syndrome, congenital disease, or serious, life-threatening condition. Defining the type of vascular lesion early and correctly is particularly important to determine the optimal approach to management and treatment of each patient. The care of pediatric patients often requires collaboration from a multitude of specialties including pediatrics, dermatology, plastic surgery, radiology, ophthalmology, and neurology. Although early characterization of vascular lesions is important, consensus guidelines regarding the evaluation and imaging of vascular anomalies does not exist to date. Here, the authors provide an overview of pediatric vascular lesions, current classification systems for characterizing these lesions, the various imaging modalities available, and recommendations for appropriate imaging evaluation. PMID:26705446

  6. Extragastric Dieulafoy's lesion

    PubMed Central

    Gauci, James; Galea, Samuel; Galea, Joseph; Schembri, Mark

    2014-01-01

    A 74-year-old man on warfarin for aortic valve replacement presented with recurrent episodes of melaena. An initial oesophagogastroduodenoscopy (OGD) was normal, as were red cell scanning and colonoscopy. It was a third OGD that revealed the cause of the melaena—a vascular lesion in the duodenum, at the junction between D1 and D2. An extragastric Dieulafoy's lesion was diagnosed, and the lesion was injected with epinephrine and tattooed. Over the following months, episodes of bleeding recurred despite further attempts at injection. Percutaneous radiologically assisted embolisation of the gastroduodenal artery, and eventually duodenotomy and oversuturing of the lesion were performed to no avail. The patient has undergone over 10 endoscopies, and has received over 70 units of packed red cells to date, since his initial presentation 6 years ago. Attempts to stop the bleeding permanently have been difficult, highlighting the complexity of managing such a lesion. PMID:25216921

  7. Primary orbital neuroblastoma with intraocular extension

    PubMed Central

    Vallinayagam, Muthukrishnan; Rao, Vasudev Anand; Pandian, Datta Gulnar; Akkara, John Davis; Ganesan, Niruban

    2015-01-01

    Neuroblastoma is an undifferentiated malignancy of primitive neuroblasts. Neuroblastoma is among the most common solid tumors of childhood. Orbital neuroblastoma is typically a metastatic tumor. In this case report, we describe a 2-year-old child with a rapidly progressing orbital tumor. Computed tomography revealed an orbital mass lesion with extraocular and intraocular components. An incisional biopsy was done, and a histopathological examination showed features suggestive of neuroblastoma. Systemic workup including ultrasonography of the abdomen, chest roentgenogram, whole body computed tomography, and bone scintigraphy showed no evidence of systemic involvement. The diagnosis of primary orbital neuroblastoma was made, and the child was subjected to chemotherapy followed by rapid melting of the tumor. Neuroblastoma should be considered in the differential diagnosis of childhood orbital tumors. PMID:26576531

  8. Cladribine and cytarabine in refractory multisystem Langerhans cell histiocytosis: results of an international phase 2 study

    PubMed Central

    Bernard, Frederic; van Noesel, Max; Barkaoui, Mohamed; Bardet, Odile; Mura, Rosella; Arico, Maurizio; Piguet, Christophe; Gandemer, Virginie; Armari Alla, Corinne; Clausen, Niels; Jeziorski, Eric; Lambilliote, Anne; Weitzman, Sheila; Henter, Jan Inge; Van Den Bos, Cor

    2015-01-01

    An international phase 2 study combining cladribine and cytarabine (Ara-C) was initiated for patients with refractory, risk-organ–positive Langerhans cell histiocytosis (LCH) in 2005. The protocol, comprising at least two 5-day courses of Ara-C (1 g/m2 per day) plus cladribine (9 mg/m2 per day) followed by maintenance therapy, was administered to 27 patients (median age at diagnosis, 0.7 years; median follow-up, 5.3 years). At inclusion, all patients were refractory after at least 1 course of vinblastine (VBL) plus corticosteroid, all had liver and spleen involvement, and 25 patients had hematologic cytopenia. After 2 courses, disease status was nonactive (n = 2), better (n = 23), or stable (n = 2), with an overall response rate of 92%. Median disease activity scores decreased from 12 at the start of therapy to 3 after 2 courses (P < .0001). During maintenance therapy, 4 patients experienced reactivation in risk organs. There were 4 deaths; 2 were related to therapy toxicity and 2 were related to reactivation. All patients experienced severe toxicity, with World Health Organization grade 4 hematologic toxicity and 6 documented severe infections. The overall 5-year survival rate was 85% (95% confidence interval, 65.2%-94.2%). Thus, the combination of cladribine/Ara-C is effective therapy for refractory multisystem LCH but is associated with high toxicity. PMID:26194764

  9. Abnormal cell surface antigen expression in individuals with variant CD45 splicing and histiocytosis.

    PubMed

    Boxall, Sally; McCormick, James; Beverley, Peter; Strobel, Stephan; De Filippi, Paola; Dawes, Ritu; Klersy, Catherine; Clementi, Rita; De Juli, Emanuella; Ferster, Aline; Wallace, Diana; Aricò, Maurizio; Danesino, Cezare; Tchilian, Elma

    2004-03-01

    Hemophagocytic lymphohistiocytosis (HLH) and Langerhans cell histiocytosis (LCH) are members of a group of rare heterogenous disorders, the histiocytoses, characterized by uncontrolled accumulation of pleomorphic infiltrates of leukocytes. The etiology of these diseases is mainly unknown. CD45 is a hemopoietic cell specific tyrosine phosphatase essential for antigen receptor mediated signaling in lymphocytes and different patterns of CD45 splicing are associated with distinct functions. Recently a polymorphism (C77G) in exon 4 of CD45 causing abnormal CD45 splicing and a point mutation affecting CD45 dimerization were implicated in multiple sclerosis in humans and lymphoproliferation and autoimmunity in mice respectively. Here we show that two patients with HLH exhibited abnormal CD45 splicing caused by the C77G variant allele, while a further 21 HLH patients have normal CD45. We have also examined 62 LCH patients and found three to have the C77G mutation. Peripheral blood thymus-derived (T) CD8(+) cells from normal individuals carrying the C77G mutation show a significant decrease in the proportion of cells expressing L-selectin and increased frequency of cells with LFA-1(hi) expression. It remains to be established whether C77G is a contributing factor in these histiocytic disorders. PMID:14630980

  10. Splenic myeloid metaplasia, histiocytosis, and hypersplenism in the dog (65 cases).

    PubMed

    Spangler, W L; Kass, P H

    1999-11-01

    Splenectomy specimens from 65 dogs with severe, diffuse, sustained, and progressive splenomegaly were examined. The clinical signs, hematology, and serum chemistry values in for the dogs were not useful diagnostic features. Microscopic changes in the spleens were distinctive and consisted of 1) myeloid metaplasia, 2) histiocytosis, 3) erythrophagocytosis, and 4) thrombosis with segmental infarction. Ultrastructural features suggested proliferative changes in the splenic reticular cells and macrophages (reticular meshwork) that described a continuum from reactive changes associated with immunologic damage of erythrocytes to neoplastic proliferation of histiocytic components. Thirty percent of the dogs survived 12 months. Approximately one half (53%) of the dogs with complete postmortem evaluations showed multiorgan involvement with a tissue distribution and cell morphology consistent with histiocytic neoplasia. For the remaining dogs (47%), only splenic pathology was consistently present, and a specific cause of death was often not evident. Distinctive histologic changes in the splenic tissues-including mitotic activity, erythrophagocytosis, giant cell formation, thrombosis/ infarction, and the proportion and distribution of histiocytic and hematopoietic cells-were statistically evaluated for prognostic relevance. The presence of giant cells was the only reliable prognostic feature, and that was indicative of a fatal outcome. These descriptive changes of myeloid metaplasia in the canine spleen are compared with the human clinical and pathologic syndromes of 1) agnogenic myeloid metaplasia, 2) hemophagocytic syndromes, and 3) hypersplenism. These diseases in humans produce histopathologic changes in the spleen that are similar to those observed in the canine splenic tissue we examined in this study. PMID:10568439