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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Immunohistochemical study on 26 cases of Langerhans cell histiocytosis (LCH) using several leukocyte antibodies in addition to traditionally used markers (S-100 protein and peanut agglutinin) revealed that the proliferating cells of LCH expressed UCHL1, MT1 as well as classically known positivity for S-100 protein, HLA-DR and peanut agglutinin but were negative for OPD4. In comparison to S-100 protein peanut agglutinin (PNA) using a two stage method produced weaker staining and positively stained cells were sparse. Also in this study, a small proportion of proliferating cells in LCH was observed to be reactive for both myeloid/macrophage antigens (KPI, MAC 387 and lysozyme) and Langerhans cell marker (S-100 protein), verifying the existence of a hybrid form of histiocytes.
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PMID:Immunohistochemical study on antigenic phenotype of Langerhans cell histiocytosis. 128 18

Four cases of benign Langerhans cell histiocytosis limited to the skin were studied. In all three self-healing cases (cases 2, 3, and 4) many dense bodies, myelin bodies, and worm bodies were found. In one chronic case (case 1) none of these was identified. In all four cases, in addition to CD1, HLA-DR, and S-100 stains, interferon-gamma and S-100 beta-subunit were positive in the dermal tumor cells. Both interferon-gamma and S-100 beta-subunits were negative in the normal epidermal Langerhans cells. A comprehensive literature review yielded 87 cases of skin-limited Langerhans cell histiocytosis. These cases could be subgrouped into three categories: (1) those that resolved spontaneously, (2) those that responded to therapy and had no recurrence, and (3) those with persistent or recurrent lesions, not responding to therapy but still limited to the skin.
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PMID:Immunohistochemistry and electron microscopy in Langerhans cell histiocytosis confined to the skin. 168 9

Histiocytosis X is very rare in late adulthood, usually taking a benign course with a good prognosis. A 56-year-old patient developed acute disseminated histiocytosis X with multiorgan involvement. Despite extensive diagnostic work-up the diagnosis could only be established on the grounds of a skin biopsy. Histiocytosis X cells are S 100 protein-positive and express the membrane antigens CD 1, CD 4 and HLA-DR. Electron microscopy reveals Birbeck granules. On static cytophotometry, histiocytosis X cells exhibited normal DNS content without aneuploidy. One year after the onset of disease the patient's condition deteriorated rapidly despite aggressive polychemotherapy (CHOP), and he died of multiorgan failure due to histiocytosis X.
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PMID:[Acute disseminated histiocytosis X (Langerhans cell histiocytosis) with fatal course in an adult]. 191 71

The cytomorphology of 13 cases of Langerhans cell histiocytosis is described. The most striking features were the presence of intranuclear clefts, pale nuclei and inconspicuous nucleoli, together with ample pale cytoplasm, only slight cellular pleomorphism, and an admixture of varying numbers of eosinophils, macrophages and degenerated cells. In 13 of 16 cases investigated ultrastructurally, characteristic Birbeck granules were detected. Out of six cases tested, four exhibited positivity for S-100, and of three cases tested, all were positive for CD1a (leu 6) and HLA-DR. In one case malignant transformation occurred, terminating in monocytic leukaemia.
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PMID:The cytology of Langerhans cell histiocytosis (histiocytosis X). 193 78

The immunophenotypic properties of the abnormal cells in routine specimens from 16 cases of Langerhans cell histiocytosis (LCH) were examined. In five cases, cryostat sections were also available. The abnormal cells expressed a similar phenotype and were positive for HLA-DR, S-100 protein, peanut agglutinin (PNA), CD1a, CD4 and several macrophage-associated markers, including CD11c, CDw32 and CD68 (the latter detectable in routine sections with antibody KP1). Staining with CD14, CD35 (C3b receptor), and CD11b (C3bi receptor) was negative with the exception of one of the cases in which a proportion of the cells showed faint positivity with CD11b. Staining for pan-T-cell (CD2, CD3, CD5) and pan-B-cell (CD19, CD22) antigens was negative in all lesions. It is concluded that LCH expresses a characteristic phenotype with some heterogeneity with regard to macrophage markers and that immunohistochemical methods in cryostat sections and routine specimens form a useful supplement to other techniques for the diagnosis of this condition.
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PMID:Immunohistochemical study of the abnormal cells in Langerhans cell histiocytosis (histiocytosis x). 210 27

In this report, we describe a patient with the classic histology of pulmonary histiocytosis X, who had bilateral reticulonodular densities and mediastinal lymph node involvement. The diagnosis was confirmed by the use of electron microscopy and immunohistochemical markers (PS100, HLA-DR, and CD1), which allowed us to recognize the lymph node infiltration of X histiocytes. An association of mediastinal lymph node enlargement with pulmonary histiocytosis X has been reported but it has never been histologically documented.
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PMID:Pulmonary histiocytosis X with mediastinal lymph node involvement. 224 Aug 46

A series of cases of histiocytosis X was studied by immunohistochemistry (20 cases), histoenzymology (5 cases) and electron microscopy (15 cases). The immunohistochemical methods showed in the majority of cells (histiocytes similar to Langerhans' cells), OKT6, HLA-DR ans S-100 protein antigens. Besides, Leu 4 antigen was also present on the surface of some cells. The histoenzymological analysis found acid phosphatase and specific esterases activities with a different repartition in histiocytes compared to macrophages. By electron microscopy, we detected the typical Birbeck granules. The signification of these granules and also the pathogenesis of the disease were discussed.
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PMID:[Histiocytosis X of the jaws. II. Structural, histo-enzymologic and immunohistochemical study]. 282 39

Clinical, morphological, ultrastructural and immunological studies were performed in a case of congenital self-healing non-Langerhans cell histiocytosis. The patient showed several aspects that have not been published before: a large nodule in the vulvar region, vesiculobullous elements and pneumonia (asymptomatic). The relationship of the vesicles and pneumonia to the histiocytic disorder is not clear. Ultrastructurally, worm-like (comma-shaped) particles, dense bodies and Birbeck granules were not found. Histiocytes were Leu-6 negative, and S100 (partly), Leu M3 and HLA-DR positive. Positive reactions were also obtained with anti-lysozyme and non-specific esterase. Several aspects of this case and of others described previously are discussed.
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PMID:Congenital self-healing non-Langerhans cell histiocytosis. 305 80

A full-term female infant was born with numerous purple firm indolent papulonodular lesions, occasionally ulcerated, ranging in size from 1 to 8 mm, scattered all over the body. The liver, spleen, lymph nodes were normal, as were the blood count, skull and chest X-ray. Biopsy of a skin lesion showed a deep dermal infiltrate consisting of histiocytic cells which were OKT6 +, HLA-DR + and S 100 +. Electron microscopy revealed numerous dense bodies, often with a myelin-like core and some Birbeck granules. The clinical course was favourable with a spontaneous regression of all lesions, sometimes through a central necrosis or atrophy, within 8 weeks. Congenital self-healing reticulohistiocytosis (CSHR) is characterized by a neonatal onset, with purple or necrotic nodular lesions showing the histopathologic aspect of histiocytosis X (although Birbeck granules are less numerous), an absence of visceral involvement and a spontaneous regression within the first 3 months of life. The place of CSHR among histiocytosis is discussed.
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PMID:[Hashimoto-Pritzker congenital self-healing histiocytosis]. 319 59

Immunohistochemical and ultrastructural evidence support the concept that histiocytosis X is the result of proliferation of pathological Langerhans' cells. Central nervous system involvement by histiocytosis X has been commonly described in multisystem disease and in association with lytic skull lesions. Unifocal brain involvement by histiocytosis X without concomitant osseous involvement is rare, with only 14 cases reported in the literature to date. Ten of these cases have involved the hypothalamus; the remaining four have involved the frontal lobe (two cases) and the temporal lobe (two cases). The fifth case of extrahypothalamic unifocal histiocytosis X, the first female case, and the first case with parieto-occipital lobe involvement, is reported. Pathology demonstrated infiltration of brain parenchyma by clusters of characteristic histiocytosis X cells with an admixture of morphologically related giant cells, eosinophils, and lymphocytes. Langerhans' granules were identified in the histiocytosis X cells by electron microscopy. Immunohistochemistry showed strong S-100 protein, HLA-DR, and T6 antigen positivity by the histiocytosis X cells. Therapy included complete surgical excision and postoperative radiation therapy for the incompletely excised lesion. Patients with unifocal extrahypothalamic histiocytosis X may have a better prognosis than patients with localized hypothalamic disease.
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PMID:Primary histiocytosis X of the parieto-occipital lobe. 325 35


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