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)

A patient who developed diffuse normolipidemic plane xanthomas also presented with IgG lambda monoclonal gammopathy, hypernephroma, an unusual family cluster of leukemia (with two family members in two generations), and a unique, acquired C1-esterase inhibitor deficiency. A second patient presented with widespread normolipidemic papular xanthomas in which histiocytes containing Langerhans' granules were found. The lipid composition of the lesions of these two patients showed striking differences. Excesses of triglycerides and cholesterol ester were demonstated in plane xanthoma, whereas phospholipids were prominent in the popular xanthoma of histiocytosis X. We present and classify these two cases to emphasize the diagnostic value of chemical and ultrastructural studies of normolipidemic cutaneous xanthomatosis.
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PMID:Unusual normolipidemic cutaneous xanthomatosis: a comparison of two cases illustrating the differential diagnosis. 7 9

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

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

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

In a female patient with histiologically ensured histiocytosis X, cell cultures modified according to NEZELOF were performed in methylcellulosis and Eagle's medium. From the 5th to the 15th day of incubation the cells were daily observed natively through a normal microscope and taken for histochemical examinations and for checking their capacity of phagocytosis. Two cell types could be distinguished: spindle-shaped fibroblasts as well as round cells of changing form and size, the alpha-naphthylacetate esterase response of which proved to be positive. The round cells can partly be regarded as HK cells, whereas giant cells could be predominantly observed during the second week. A phagocytosis of iron chips and human erythrocytes loaded with antibodies could be revealed in both types. HK cells represented mature macrophages, from which giant cells developed under in vitro conditions. There is still not explanation as to the local and immunological factors characterizing the clinical picture of histiocytosis X.
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PMID:[In vitro studies of cells in histiocytosis X]. 618 82

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

Hodgkin's disease (HD) shows rare neoplastic Hodgkin and Reed-Sternberg cells embedded in an abundant reactive infiltrate containing, among other cell types, neutrophilic granulocytes. Interleukin (IL)-8 is chemotactic for neutrophils. The expression of IL-8 was tested by in situ hybridization with 35S-labeled IL-8-specific RNA probes on 38 cases of HD. Reactive lesions, non-Hodgkin's lymphomas of B and T phenotype, and Langerhans cell histiocytosis served as controls. IL-8 expression was observed in Hodgkin and Reed-Sternberg cells in 3 of 33 cases of classical HD and in reactive cells in 20 of 33 HD cases as evidenced by combined isotopic in situ hybridization and immunohistology for the demonstration of cell-type-characteristic antigens or enzyme histochemistry for chloroacetate esterase. IL-8-positive cells were more numerous in cases of nodular sclerosing HD as compared with the mixed cellularity histotype (P = 0.01). The number of IL-8-positive cells and the density of neutrophils were positively correlated (P < 0. 01). In 5 cases of lymphocyte-predominant HD, IL-8 expression was not displayed. Non-Hodgkin's lymphoma cases contained IL-8 transcripts only in 1 of 23 cases in sparse reactive cells. In 4 of 7 cases of Langerhans cell histiocytosis, IL-8-specific signals were displayed in S100-negative cells. In conclusion, IL-8 expression in HD is largely confined to reactive cells and associated with infiltration by neutrophils. Elaboration of other cytokines by Hodgkin and Reed-Sternberg cells and reactive cells may explain the frequent expression of this cytokine in HD, particularly in the nodular sclerosing type.
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PMID:Interleukin-8 in Hodgkin's disease. Preferential expression by reactive cells and association with neutrophil density. 864 63

Pulmonary Langerhans' cell histiocytosis (PLCH) is a disease characterized by the occurrence of complex fibro-cellular interstitial lesions dominated by Langerhans' cells (LC), which occurs predominantly in young adult smokers. We undertook this retrospective study to better define the lymphohistiocytic cell populations in PLCH in order to obtain a greater insight into its pathogenesis. Formalin-fixed, paraffin-embedded, surgically excised, archival lung tissue from seven patients (two males, five females; average age 34.9 years) was immunostained with a panel of antibodies for lymphohistiocytic markers: CD1a, CD3, CD4, CD8, CD15, CD20, CD56, TIA-1, CD68-PGM1, Mac387, and mast cell tryptase. Double immunolabeling was performed with CD1a/Mac387. Leder cytochemical stain for chloroacetate esterase was also performed. A moderate number of lymphocytes, predominantly T lymphocytes, were scattered diffusely within the lesions. The mean CD4/CD8 ratio was 0.1/1. The CD3/CD8 ratio (1.18/1) substantiated the CD4/CD8 ratio. The CD8 subset was CD56-negative and TIA-1-positive, indicating a cytotoxic T lymphocyte phenotype. CD68-PGM1 was strongly positive in alveolar macrophages (AM) and weakly stained LC. Mac387, a marker of activated macrophages, weakly stained AM, while highlighting other interstitial cells. These interstitial cells appeared not to be LC (substantiated by CD1a/Mac387 dual labeling) or CD68-PGM-1-positive macrophages. Having excluded mast cells (positive with mast cell tryptase) and neutrophils (positive with CD15 and Leder stains), there appeared to be a residual population of non-Langerhans cell monocytoid cells (NLMC), which were Mac 387+, CD68-PGM1-, Mast cell tryptase-, CD15-, and CD1a-. Our results showed a predominance of CD8+, TIA-1+ cytotoxic T lymphocytes among the lymphocyte subsets which appear to interact with LC and AM in PLCH lesions. A small sub-population of NLMC was also present. Further studies are required to better define and to evaluate the role of cytotoxic T cells and NLMC in the pathogenesis of PLCH.
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PMID:Lymphocyte sub-populations and non-Langerhans' cell monocytoid cells in pulmonary Langerhans' cell histiocytosis. 1833 20