Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Skin biopsies and a lymphnode of three children with infantile Histiocytosis-X (Letter-Siwe Disease) were studied with enzymehistochemical and sheep-erythrocyte rosetting techniques. The majority of cells making up the infiltrates of skin and lymphnode showed rather weak acid phosphatase and nonspecific esterase activity but considerable leucyl-beta-naphtylamidase activity. Sheep-erythrocyte rosetting techniques performed on frozen sections indicated the presence of receptors for the Fc fragment of IgG, but no receptors for C3 could be demonstrated. Cells with the same enzymehistochemical characteristics could be found in thymus-dependent areas of normal spleen, of normal and reactive lymphnodes and in thymic medulla but not in B-cell areas or thymic cortex. It is suggested that Histiocytosis-X cells belong to the Mononuclear Phagocyte System and that they are related to or identical with cells normally present in the thymus dependent areas of the lymphoid tissue involved with the functioning of cell-mediated immunity.
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PMID:Infantile histiocytosis X. 15 Mar 6

Characteristic mononuclear cells with distorted nuclei and abundant pale-staining cytoplasms together with osteoclast-like giant cells are the cytologic hallmarks of histiocytosis X. The demonstration of acid phosphatase within giant cells in paraffin-embedded sections is a valuable aid to diagnosis. The histologic differential diagnosis of histiocytosis X includes some allergic granulomas, Hodgkin's disease, myelomonocytic leukemia, mastocytosis, and malignant histiocytosis. Some technical prerequisites for accurate diagnosis of histiocytosis X are considered briefly.
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PMID:Subtle clues to diagnosis by histochemistry. Histiocytosis X. 31 20

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

A series of human multinucleate giant cells (MGCs) of the endocytotic type were studied using enzyme histochemical methods for dehydrogenases, glycosidases, phosphatases, and peptidases. Several enzyme patterns were found. The subgroup of MGCs associated with inflammatory granulomatous processes (sarcoidosis, granulomatous myositis, familial granulomatosis, lymphogranuloma, granulomatous cholangitis) was characterized by high activities of nonspecific esterase (NE) and tartrate-sensitive acid phosphatase (AcPase-Ts). There was no detectable activity of peptidases or tartrate-resistant isoenzyme of acid phosphatase (AcPase-Tr). This enzyme equipment was indistinguishable from that in mononuclear precursors in the granulomas. The other MGCs of the series displayed enzyme patterns substantially different from their monocytic precursors (blood monocytes and Langerhans cells). The subgroup of foreign body associated MGCs (resorption of fat, keratin, and suture material) was characterized by high activities of NE, AcPase-Tr, and greatly variable activities of both peptidases studied. The latter lacked predilection for certain subcellular regions. The subgroup of osteoclasts and so-called giant cell tumours (osteoclastoma, giant cell tumour of soft parts, giant cell epulis of peripheral, and central types) displayed very low activity of NE, high activity of AcPase-Tr, and strong activities of peptidases. The latter were localized near the surface membrane of the polykarya. MGCs in histiocytosis X (HX) differed from the previous group by higher values of NE in average. All MGC types had common denominator in the absence of alkaline phosphatase activity, on average intense dehydrogenase activities, mostly low beta-glucuronidase and highly variable alpha-mannosidase activities. The enzyme pattern heterogeneity is discussed with regard to the phenomenon of enzyme induction and depression occurring in course of polykaryon production. The variability of phenomenon may reflect reactive adaptation to varying functional demands imposed on MGCs under different conditions.
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PMID:Enzyme patterns in human endocytotic multinucleate giant cells--a histochemical study. 287 82

The immunological phenotype of the cells involved in skin and lymph node lesions from two cases of histiocytosis X (H-X) were analysed by immunofluorescence techniques using combinations of heterologous and monoclonal antisera to Ia-like antigen and human cortical thymocyte (HTA-1) determinant. These cells were also characterised by a new technique using simultaneous immunofluorescence and enzyme histochemistry for acid phosphatase (ACPase). The major cell type in the lesions was found to express the same Ia+, HTA-1+ phenotype as normal epidermal Langerhans' cells (LC) and was unreactive for ACPase. Additional cell types included Ia-, HTA-1- multinucleate giant cells and residual lymphoid populations. These findings endorse previous concepts that H-X is a proliferation of abnormal LC and emphasise the heterogeneous nature of the cells involved in the disease.
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PMID:Combined immunological and histochemical analysis of skin and lymph node lesions in histiocytosis X. 617 64

A 9-year-old girl had a widespread papular eruption that was histologically characterized by benign histiocytes that were acid phosphatase-positive, but that lacked Langerhans' granules ultrastructurally. New lesions had continued to develop since the patient was 3 months old, and individual lesions had resolved over a period of two to three months. The histopathologic, histochemical, and ultrastructural findings in this patient were similar to those previously described for eruptive histiocytomas in adults. Papular histiocytomas in childhood may be found in histiocytosis X, reticulohistiocytoma, xanthogranuloma, xanthoma disseminatum, or eruptive histiocytoma. These diseases may be differentiated on the basis of differing histiocytic populations and the clinical courses of the lesions in each disorder.
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PMID:Eruptive histiocytoma of childhood. 624 40

The proliferating cell in histiocytosis X has been thought to be an abnormal Langerhans cell since the identification in 1965 of the characteristic X-bodies morphologically identical to the Birbeck granules of epidermal Langerhans cells. This conclusion is based primarily on this finding and on morphologic similarities at the light microscopic level. Enzyme histochemical data have been somewhat conflicting and have not provided strong support for this conclusion. Recently, immunohistochemical studies have shown data consistent with a Langerhans cell origin for histiocytosis X cells. This study documents the presence of nonspecific esterase, acid phosphatase, and adenosine triphosphatase in both Langerhans cells and histiocytosis X cells. Both types of cells also react with antibodies directed against widely distributed leukocyte antigens (HLA-A,B,C; Ia; L3B12); Langerhans cell/thymocyte antigen [Leu 6(T6)]; histiocyte antigen (Leu-M3); helper T-cell/histiocyte antigen [Leu-3(T4)]; and S-100 protein. These results complement earlier immunologic studies and add enzyme histochemical data that strongly support the concept of histiocytosis X as a proliferative disorder of cells of Langerhans lineage.
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PMID:Histiocytosis X cells and Langerhans cells: enzyme histochemical and immunologic similarities. 638 Dec 83

9 cases of malignant histiocytosis (3 males, 6 females, age 30 to 83 years) have been observed in the biopsy and autopsy material of the Institute of General Pathology and Pathological Anatomy of the Medical Academy of Erfurt, GDR. Malignant histiocytosis which derives from tissue macrophages/histiocytes is characterized by a proliferation of non-cohesive atypical histiocytes mainly in lymph nodes, spleen, liver, and bone marrow. The tumor cells are large, pleomorphic and show an irregularly-shaped, cleaved and often kidney-like nucleus with 1-3 medium-sized nucleoli. The cytoplasm is moderately basophilic and grey-blue when stained with azure B eosin or according to Pappenheim. Diffuse cytoplasmic activities of nonspecific esterase and acid phosphatase are further properties. Another sign of malignant histiocytic cells should be stressed, i.e., the diffuse intracytoplasmic perinuclear PAS-positive spot which hitherto has not been mentioned in the literature. Morphological variants of malignant histiocytosis are fibrosing (2 cases) and erythrophagocytosing types (2 cases). Reactive histiocytosis, histiocytosis X and malignant non Hodgkin-lymphomas, esp. immunoblastic lymphomas, are to be taken into account in differential diagnosis.
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PMID:[Contribution to morphology of malignant histiocytosis (author's transl)]. 709 May 92