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Cell Histiocytosis (cell + histiocytosis)
Kinds of Cell Histiocytosis Selected AbstractsLangerhans Cell Histiocytosis: A Review of the Current Recommendations of the Histiocyte SocietyPEDIATRIC DERMATOLOGY, Issue 3 2008Elizabeth K. Satter M.D., M.P.H. Historically, the nomenclature regarding this entity has been confusing because the disease had been subcategorized simply based upon the different clinical manifestations. In the following article, we summarize the current recommendation of the Histiocyte Society regarding the classification, evaluation, prognosis, and treatment of Langerhans cell histiocytosis. [source] Urticating Hashimoto,Pritzker Langerhans Cell HistiocytosisPEDIATRIC DERMATOLOGY, Issue 1 2001David F. Butler M.D. The Darier sign has been a reliable feature in the diagnosis of mastocytosis. However, the cutaneous infiltrate of Hashimoto,Pritzker Langerhans cell histiocytosis (LCH) may contain a large number of mast cells, leading to confusion both clinically and histologically. We report an infant who developed red-brown papules of Hashimoto,Pritzker LCH during the neonatal period and presented with a positive Darier sign and acute urticaria. [source] Smoking preceded pulmonary involvement in adults with Langerhans cell histiocytosis diagnosed in childhoodACTA PAEDIATRICA, Issue 11 2000C Bernstrand ABSTRACT. Two patients with childhood Langerhans cell histiocytosis (LCH) (aged 2 and 6 y at diagnosis) in whom pulmonary involvement was diagnosed in adulthood, 23 and 12 y later, respectively, are presented. In each patient, smoking preceded the diagnosis of pulmonary involvement by 3 y, providing further evidence that smoking is a risk factor in the development of pulmonary LCH. ,Langerhans cell histiocytosis, lungs, smoking [source] Langerhans cell histiocytosis in lymph nodes , cytomorphological diagnosis and pitfallsCYTOPATHOLOGY, Issue 6 2000S. Kakkar Background Langerhans cell histiocytosis (LCH) is a rare disorder of unknown aetiology that may present as a multisystem or unisystem disease. The Lymph nodes can be involved as part of disseminated disease, as a metastatic site draining a focus of LCH or may be a unisystem involvement. Paucity of literature on the cytomorphology of LCH in lymph nodes led us to undertake this study. Materials and methods Nine cases with a confirmed histological diagnosis of LCH and a prior lymph node aspirate were retrieved over a 12 year period (1988,1999). Five more cases were reviewed where the cytological diagnosis of LCH was rendered on a background of clinical and radiological findings. Papanicolaou and May Grunwald,Giemsa-stained smears were examined. S-100 protein staining was available in four cases. Results and conclusions Nine cases had multisystem involvement, while in five cases only lymph nodes were involved. There were eleven males and three females; age ranged from five months to 27 years. The cytological diagnosis of LCH had been rendered in six, suspected in four and missed in four. Of the latter, two were reclassified as LCH on review, one as reactive lymphadenitis and in one a necrotising lesion was suspected. The pathognomonic ,LCH cell' was identified in 12 of 14 cases along with varying numbers of eosinophils, polymorphs and lymphocytes. Giant cells were seen in only six cases. In conclusion lymph node involvement by LCH can be identified on aspirates. However, LCH must be differentiated from dermatopathic lymphadenitis, sinus histiocytosis with massive lymphadenopathy and Hodgkin's disease. [source] Adult Langerhans cell histiocytosisEUROPEAN JOURNAL OF HAEMATOLOGY, Issue 5 2006Marcus Stockschlaeder Abstract:, Langerhans cell histiocytosis (LCH) is a proliferative histiocytic disorder of unknown cause originating from dendritic cells. The clinical presentation of LCH is highly variable. Although the features of this disease have been well described in children, they remain poorly defined in adults. Here, we review the current knowledge about adult LCH, focussing on clinical presentation, diagnosis, treatment, and prognosis. [source] A colonic polyp due to Langerhans cell histiocytosis: a lesion not to be confused with metastatic malignant melanomaHISTOPATHOLOGY, Issue 4 2006S Sharma No abstract is available for this article. [source] Langerhans cell histiocytosis: fascinating dynamics of the dendritic cell,macrophage lineageIMMUNOLOGICAL REVIEWS, Issue 1 2010R. Maarten Egeler Summary:, In its rare occurrence, Langerhans cell histiocytosis (LCH) is a dangerous but intriguing deviation of mononuclear phagocytes, especially dendritic cells (DCs). Clinically, the disease ranges from self-resolving or well manageable to severe and even fatal. LCH lesions in skin, bone, and other sites contain high numbers of cells with phenotypic features resembling LCs admixed with macrophages, T cells, eosinophils, and multinucleated giant cells. Here we review current progress in the LCH field based on two central questions: (i) are LCH cells intrinsically aberrant, and (ii) how does the lesion drive pathogenesis? We argue that LCH cells may originate from different sources, including epidermal LCs, tissue Langerin+ DCs, or mononuclear phagocyte precursors. Current and prospective in vitro and in vivo models are discussed. Finally, we discuss recent insights into plasticity of T-helper cell subsets in light of the lesion microenvironment. LCH continues to provide urgent clinical questions thereby inspiring innovative DC lineage research. [source] Unifocal Langerhans cell histiocytosis of the oral mucosaJOURNAL DER DEUTSCHEN DERMATOLOGISCHEN GESELLSCHAFT, Issue 7 2009Susanna Fistarol Summary A 24-year-old man was admitted for a painful gingival ulcer. Histology and immunohistochemistry of a lesional biopsy revealed the diagnosis of Langerhans cell histiocytosis (LCH). To rule out multifocal disease, a complete staging was performed. There was no evidence of bony lesions or any other organ involvement. The diagnosis of LCH restricted to the oral mucosa was established. The complete oral lesion was ablated by CO2 laser and subsequently treated topically with triamcinolone acetonide. The patient is still in remission after one year of follow-up. LCH confined to the oral mucosa is rare. It presents usually as an inflammatory or ulcerative lesion, easily leading to misinterpretation and delayed diagnosis. Patients with limited unifocal mucocutaneous disease, as in the present case, usually have an excellent prognosis. However, the oral lesion may represent an early sign of LCH, predating and progressing to an aggressive life-threatening multiorgan disease. [source] Fibroblastic rheumatism: fibromatosis rather than non-Langerhans cell histiocytosisJOURNAL OF CUTANEOUS PATHOLOGY, Issue 5 2010Nicolas Kluger Background: Fibroblastic rheumatism is a unique fibro-proliferative disease affecting the skin and joints. It is characterized by distinctive clinical and histological features related to benign spindle-shaped cells proliferation. Pediatric reports are scarce in the literature. Objective: We describe here a new case in a 10-year-old boy and discuss the potential origin of the cell proliferation. Methods: Clinical findings, radiology, microscopic examination and outcome are reviewed. Histopathology and immunochemistry studies were performed on skin biospies using CD68, CD163, desmin, factor XIIIa, CD34, smooth muscle actin, PS100, epithelial membrane antigen, and calponin. Results: Histological sections disclosed a rather circumscribed nonencapsulated nodular infiltrate, invading the dermis and the upper subcutaneous tissue, consisted of a proliferation of spindle or stellate-shaped cells and thickened collagen fibers. Orcein staining showed disappearance of the elastic network. Aponeurosis and muscle were normal. A mild perivascular lymphohistiocytic infiltrate was noted. Calponin-staining was less strongly expressed as SMA, and some of them but not all were CD68 positive, as well. On the other hand, all were CD34, CD163, FXIIIa, PS100, EMA and desmin-negative. Conclusion: The true origin of these cells remains unclear. Some authors have speculated a histiocytic origin. However, immuno-chemical staining in our case failed to confirm this hypothesis and instead supported a fibroblastic/myofibroblastic origin. Given the clinical course and the histological and immunohistochemical results, we suggest that FR should be added to the group of fibromatoses. Kluger N, Dumas-Tesici A, Hamel D, Brousse N, Fraitag S. Fibroblastic rheumatism: fibromatosis rather than non-Langerhans cell histiocytosis. [source] Prominent Langerhans' cell migration in the arthropod bite reactions simulating Langerhans' cell histiocytosisJOURNAL OF CUTANEOUS PATHOLOGY, Issue 12 2007Se Hoon Kim Background:, Epidermal Langerhans' cells (LCs) play pivotal roles in cutaneous immune responses. An encounter with antigens or other stimuli causes the mobilization and migration of LCs. Therefore, some dermatoses, which originated from antigenic stimuli or trauma, can show LC migration. Recently, we experienced several cases of anthropod bites that showed marked inflammatory infiltrates with eosinophils and CD1a-positive LCs. It was difficult to differentiate these cases from Langerhans' cell histiocytosis (LCH). Methods:, The degree and pattern of LC infiltration in the skin of arthropod bite reaction was evaluated. The characteristics of CD1a immunohistochemical expression in the arthropod bite reactions were compared with those of LCH. Results:, A few arthropod bite cases (approximately 36%) showed extensive CD1a-positive LCs in the dermis, especially in the perivascular area. In addition, the CD1a expression patterns of LCs in the arthropod bite reactions were dendritic, whereas that of tumor cells in LCH were distinctly membranous and cytoplasmic. Conclusion:, Some arthropod bite reactions can show marked CD1a-positive LCs in the dermis, especially in the perivascular area. The dendritic CD1a immunohistochemical staining pattern in arthropod bite reactions is useful in helping to differentiate from LCH. [source] Lymphomatoid papulosis with CD1a+ dendritic cell hyperplasia, mimicking Langerhans cell histiocytosisJOURNAL OF CUTANEOUS PATHOLOGY, Issue 7 2007Chris H. Jokinen Although CD1a+ dendritic cells (DC) in cutaneous T-cell lymphomas (CTCL) have been well documented, the presence of large numbers of DC within lymphoid infiltrates can pose a diagnostic difficulty. We present a case of a 70-year-old man with a 3-year history of recurrent red papules and plaques on the extremities and trunk that was referred to our institution, with the diagnosis of Langerhans cell histiocytosis. Skin biopsies showed a wedge-shaped cellular infiltrate in the superficial and deep dermis consisting of two cell populations. Most prominent were clusters of epithelioid cells with grooved nuclei and abundant eosinophilic cytoplasm, which stained with antibodies to CD1a and S-100. A second, less prominent population of atypical lymphocytes, some with enlarged, hyperchromatic and convoluted nuclei, were intermixed. The latter were positive for CD30, CD3 and CD5 and negative for CD20, CD34, CD68, ALK-1 and TdT. T-cell receptor gene rearrangement studies confirmed a clonal T-cell population, which with the clinical history was consistent with the diagnosis of lymphomatoid papulosis. While previous studies have shown an increased density of dermal DC in CTCL, we believe that this represents the first report of an unusually florid DC proliferation mimicking Langerhans cell histiocytosis and masking a lymphoproliferative disorder. [source] IL-1, and PGE2 levels are increased in the saliva of children with Langerhans cell histiocytosisJOURNAL OF ORAL PATHOLOGY & MEDICINE, Issue 9 2008Virginia F. Preliasco Langerhans cell histiocytosis (LCH) is a rare disorder mainly of children, whose pathogenesis is still unknown. Some studies have demonstrated that LCH lesions produce different cytokines abnormally that may be relevant to the pathogenesis of the disease. The purpose of this study was to investigate interleukin-1, (IL-1,) and prostaglandin E2 (PGE2) levels in saliva from children with different clinical subtypes of LCH. We studied 29 children with LCH: seven unifocal (Group I), seven multifocal (Group II), 15 multisystemic (Group III) and 12 healthy volunteers (Group IV). Salivary IL-1, and PGE2 levels were significantly higher in LCH than in normal children. A multi-comparison test showed significantly (P < 0.001) higher levels of both IL-1, and PGE2 in saliva from Group III compared with Groups II and I. A significant correlation (r = 0.05) between IL-1, and PGE2 concentrations in saliva from each group was determined. Our findings demonstrated an association between high concentrations of salivary IL-1, and PGE2 and advanced stages of the disease. This allows us to suggest that the abnormal amount of these factors in saliva may serve as a risk marker for disease progression. [source] Langerhans cell histiocytosis and human herpes virus 6 (HHV-6), an analysis by real-time polymerase chain reactionJOURNAL OF ORTHOPAEDIC RESEARCH, Issue 3 2006Michael P. Glotzbecker Abstract Patients with Langerhans cell histiocytosis (LCH) usually present to orthopedic surgeons because this disease most commonly affects bone. The pathogenesis of LCH is unknown, although roles for environmental, infectious, immunologic, and genetic causes have been postulated. More specifically, there is limited data suggesting that human herpes virus 6 (HHV-6) may be a potential etiologic agent. Frozen biopsy material was obtained from 13 patients with LCH and 20 patients without the disease. After ensuring histologic adequacy of the material, the tissue was tested for HHV-6 by qualitative and quantitative real-time TaqMan PCR. Four of 13 patients with LCH had evidence of HHV-6 DNA in their tissue while 7 of 20 control patients tested positive for HHV-6 genome. Viral loads are reported for the positive patients; no statistical difference was observed in the presence or quantity of HHV-6 DNA found in either population, suggesting that the prevalence of HHV-6 in the tissue of LCH patients is the same as that found in tissue from individuals without disease. © 2005 Orthopaedic Research Society. Published by Wiley Periodicals, Inc. J Orthop Res 24:313,320, 2006 [source] Langerhans cell histiocytosis: oral/periodontal involvement in adult patientsORAL DISEASES, Issue 8 2009S Annibali Objective:, Langerhans cell histiocytosis (LCH) is a clonal proliferative multisystem disease. Although bone and mucosae have been classified as non-risk organs, their involvement may increase the risk of disease progression. Oral and periodontal lesions are burdened with a significant impairment of quality of life for associated signs, symptoms and loss of function. Most of information regards paediatric disease; the disease in adults has received limited attention. Subjects and Methods:, A total of 31 adult patients affected by immuno-histopathology confirmed LCH have been prospectively examined; attention was paid to the occurrence and characterization of oral lesions. Results:, Twelve patients developed oral lesions. Posterior regions of jawbones were always affected; the involvement of anterior regions was not constant. Unifocal oral involvement was significantly associated with multisystemic disease while multifocal lesions were associated with unisystemic disease. Oral disease presented with soft tissue ulcers (50% of cases), gingival bleeding (66.7%), pain (83.4%), periodontal damage (50%), tooth mobility (16.7%), non-healing extraction socket (8.3%); 41.6% of patients complained of negative outcomes on quality of life. Oral lesions were easily handled with local measures. Conclusions:, Posterior regions require attention; single oral lesions may be part of multisystemic disease; oral and periodontal lesions may be early signs of disease reactivation. [source] Langerhans cell histiocytosis with digestive tract involvement,PEDIATRIC BLOOD & CANCER, Issue 4 2010Satya P. Yadav DCH Abstract Gastrointestinal tract (GIT) involvement in Langerhans cell histiocytosis (LCH) is not commonly described. We present two children presenting with GIT involvement with LCH, one successfully treated on standard protocol and other being treated on a protocol for relapsed disease. A review of literature showed almost 95% children were less than 2 years of age and 62% were females. Vomiting, abdominal pain, constipation, intractable diarrhea, malabsorption, bloody stools, protein-losing enteropathy, and even intestinal perforation are some of the reported symptoms. More than 50% patients died within 18 months from diagnosis. Pediatr Blood Cancer. 2010;55:748,753. © 2010 Wiley-Liss, Inc. [source] Langerhans cell histiocytosis following acute leukemia in an adult,AMERICAN JOURNAL OF HEMATOLOGY, Issue 10 2009Rebecca Hirsh No abstract is available for this article. [source] An infant with self-healing cutaneous Langerhans cell histiocytosis followed by isolated thymic relapsePEDIATRIC BLOOD & CANCER, Issue 2 2009Naoki Hatakeyama MD Abstract Thymic involvement with Langerhans cell histiocytosis (LCH) typically occurs in children as part of multi-system (M-S) LCH. Patients who develop skin-only LCH during infancy may either follow a self-healing course with spontaneous regression or may progress to M-S involvement. We describe a male infant who developed isolated thymic LCH after spontaneous complete regression of isolated cutaneous lesions. His erythrocyte sedimentation rate and C-reactive protein increased temporarily during the skin-only stage of LCH, and increased again considerably during the thymic relapse. Even for patients with skin-only LCH, these laboratory data might indicate possible relapse or late progression of the disease. Pediatr Blood Cancer 2009;53:229,231. © 2009 Wiley-Liss, Inc. [source] Isolated pulmonary Langerhans cell histiocytosis with recurrent bilateral pneumothoraces treated with chemotherapy and chemical pleurodesisPEDIATRIC BLOOD & CANCER, Issue 1 2009Lubbna Valliani MD No abstract is available for this article. [source] Central nervous system-related permanent consequences in patients with Langerhans cell histiocytosis,PEDIATRIC BLOOD & CANCER, Issue 1 2007Edda Mittheisz MD Abstract Background Permanent consequences in Langerhans cell histiocytosis (LCH) are irreversible late sequelae related to the disease that may severely impair the quality of life of survivors. The frequency and pattern of permanent consequences affecting the central nervous system (CNS) remains to be determined. Procedure In this single center study, 25 LCH patients observed for a median time of 10 years 3 months underwent a uniform thorough follow-up program including neuropsychological testing and electrophysiological evaluation. Results Overall permanent consequences were seen in 9 of 25 patients. Intracranial abnormalities were the most frequent including diabetes insipidus (DI) in seven patients, anterior pituitary deficiencies in five patients, and neurodegenerative CNS disease in five patients. No patient had overt neurological symptoms upon neurological evaluation, but psychological testing revealed subtle deficits in short-term auditory memory (STAM) in 14 patients. Brain stem evoked potentials showed abnormalities in four of nine tested patients, all of these four had neurodegeneration on MRI. Conclusion Psychoneuroendocrine sequelae were found in an unexpectedly high number of patients in this single center study. Long-term follow-up focusing on such sequelae are important in LCH survivors, in order to detect early deficits, to monitor the evolution of the disease, and to provide specific support. Pediatr Blood Cancer 2007;48:50,56. © 2006 Wiley-Liss, Inc. [source] Langerhans cell histiocytosis affecting the eyesPEDIATRIC BLOOD & CANCER, Issue 5 2006Giulio J. D'Angio MD No abstract is available for this article. [source] Langerhans cell histiocytosis: Too many cytokines, not enough gene regulation?PEDIATRIC BLOOD & CANCER, Issue 2 2006Maurizio AricòArticle first published online: 12 JAN 200 No abstract is available for this article. [source] Langerhans Cell Histiocytosis: A Review of the Current Recommendations of the Histiocyte SocietyPEDIATRIC DERMATOLOGY, Issue 3 2008Elizabeth K. Satter M.D., M.P.H. Historically, the nomenclature regarding this entity has been confusing because the disease had been subcategorized simply based upon the different clinical manifestations. In the following article, we summarize the current recommendation of the Histiocyte Society regarding the classification, evaluation, prognosis, and treatment of Langerhans cell histiocytosis. [source] Urticating Hashimoto,Pritzker Langerhans Cell HistiocytosisPEDIATRIC DERMATOLOGY, Issue 1 2001David F. Butler M.D. The Darier sign has been a reliable feature in the diagnosis of mastocytosis. However, the cutaneous infiltrate of Hashimoto,Pritzker Langerhans cell histiocytosis (LCH) may contain a large number of mast cells, leading to confusion both clinically and histologically. We report an infant who developed red-brown papules of Hashimoto,Pritzker LCH during the neonatal period and presented with a positive Darier sign and acute urticaria. [source] Hematopoietic stem cell transplantation in Langerhans cell histiocytosis: Case report and review of the literaturePEDIATRIC TRANSPLANTATION, Issue 3 2009Vural Kesik Abstract:, The prognosis in children with LCH who do not respond to the conventional therapies is very poor. SCT may be a new approach. However, there are limited data about the results of the transplantations. Herein we report a patient with refractory multisystem LCH who underwent allogeneic bone marrow transplantation and is disease and treatment free 54 months after transplantation. Further studies are required to establish the role of SCT in refractory LCH. [source] Efficacy and safety of linezolid in immunocompromised children with cancerPEDIATRICS INTERNATIONAL, Issue 5 2010Maria Moschovi Abstract Background:, The aim of this study was to determine the safety, tolerance and efficacy of linezolid for the treatment of infections from Gram-positive bacteria in immunocompromised children with cancer. Methods:, This was a prospective non-comparative unblinded study in the Hematology/Oncology Unit over a two-year period, administering linezolid as monotherapy in children with cancer. Results:, Seventeen children received linezolid (30 mg/kgr: 3 i.v. per day). Mean duration of linezolid administration was 12.2 days (range, 6,38 days), while the median age of the evaluable patients was 2.2 years (range, 6 months,11.2 years). Primary diagnosis was acute lymphoblastic leukemia (nine patients), brain tumor (three patients), multi-organ Langerhans cell histiocytosis (two patients), rhabdomyosarcoma, Burkitt's lymphoma and ovarian tumor (one patient each). All patients were in the midst of chemotherapy cycles. Ten out of 17 children had positive blood cultures (methicillin-resistant Staphylococcus aureus, four patients; vancomycin-resistant Enterococcus, three patients; penicillin-resistant Streptococcus pneumoniae, three patients), while seven of the 17 had fever and vancomycin-resistant Enterococcus in stool cultures. All patients were considered clinically cured after the end of the linezolid regimen (100% efficacy). The main adverse events were thrombocytopenia grade 1,3 and anemia grade 2,3 (four and two patients, respectively). Chemotherapy-induced myelotoxicity (six patients) was not worsened during linezolid therapy. No bleeding episodes were presented. Self-limited diarrhea grade 1,2 was presented in four patients (mean duration 2 days). The total adverse event rate was 23.5%; however, there was no premature cessation of linezolid in any patient. Conclusions:, Linezolid may be another effective and safe therapy to treat infections from resistant Gram-positive bacteria in immunocompromised children, even in young ages. [source] 18F-fluorodeoxyglucose,positron emission tomography scanning is more useful in followup than in the initial assessment of patients with Erdheim-Chester diseaseARTHRITIS & RHEUMATISM, Issue 10 2009Laurent Arnaud Objective Erdheim-Chester disease (ECD) is a rare form of non,Langerhans' cell histiocytosis. The aim of this study was to assess the value of whole-body scanning with 18F-fluorodeoxyglucose,positron emission tomography (FDG-PET) in a large cohort of ECD patients from a single center. Methods We retrospectively reviewed all PET scans performed on 31 patients with ECD who were referred to our department between 2005 and 2008. PET images were reviewed by 2 independent nuclear medicine specialist physicians and were compared with other imaging modalities performed within 15 days of each PET scan. Results Thirty-one patients (10 women and 21 men; median age 59.5 years) underwent a total of 65 PET scans. Twenty-three patients (74%) were untreated at the time of the initial PET scan, whereas 30 of the 34 followup PET scans (88%) were performed in patients who were undergoing immunomodulatory therapy. Comparison of the initial and followup PET scans with other imaging modalities revealed that the sensitivity of PET scanning varied greatly among the different organs studied (range 4.3,100%), while the specificity remained high (range 69.2,100%). Followup PET scans were particularly helpful in assessing central nervous system (CNS) involvement, since the PET scan was able to detect an early therapeutic response of CNS lesions, even before magnetic resonance imaging showed a decrease in their size. PET scanning was also very helpful in evaluating the cardiovascular system, which is a major prognostic factor in ECD, by assessing the heart and the entire vascular tree during a single session. Conclusion The results of our large, single-center, retrospective study suggest that the findings of a FDG-PET scan may be interesting in the initial assessment of patients with ECD, but its greater contribution is in followup of these patients. [source] Langerhans cell histiocytosis: a case study suggesting a reactive aetiologyBRITISH JOURNAL OF DERMATOLOGY, Issue 1 2010E. Le Fourn No abstract is available for this article. [source] Cutaneous Langerhans cell histiocytosis in an elderly man successfully treated with narrowband ultraviolet BBRITISH JOURNAL OF DERMATOLOGY, Issue 6 2007S. Imafuku No abstract is available for this article. [source] S100-, serum protein,a new marker in the diagnosis and monitoring of Langerhans cell histiocytosis?BRITISH JOURNAL OF DERMATOLOGY, Issue 1 2000S. Ugurel No abstract is available for this article. [source] Choroidal Langerhans' cell histiocytosisACTA OPHTHALMOLOGICA, Issue 1 2000In Taek Kim ABSTRACT. Purpose: To report a patient with choroidal Langerhans' cell histiocytosis. Methods: A solitary tumor was found in the left eye of a 49-year-old male who had no definite history of systemic disorders, but had observed visual disturbances for a period of 1 month. Ultrasonography, fluorescein angiography, and indocyanine green angiography were performed and the eyeball was enucleated. We prepared the specimen for microscopic examinations. Results: Fluorescein angiographic findings of the lesion were mottled hyperfluorescence in the arteriovenous phase and strong hyperfluorescence in the late phase. Hypofluorescence in both early and late phases showed on indocyanine green angiogram. The lesion of choroid was widely infiltrated by histiocytes, though no extraocular invasion was found. Immunohistochemical studies including S-100 and CD 68 staining revealed characteristic features of Langerhans' cell histiocytosis. Electron microscopic examination of the histiocytes showed histiocytosis X body (Birbeck granule) in the cytoplasm and indented nucleus. Conclusion: We consider that this is a case of choroidal Langerhans' cell histiocytosis with no evidence of systemic lesions. [source] | ||||||||||||||||||||||||||||