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 histochemical study of enzymatic activities was undertaken in five cases of histiocytosis X (two localized bone forms, two generalized forms, and one involving mainly the skin), each of which revealed characteristic structural features at the optical and ultrastructural levels. A confirmation was made of the original assumption of high acid alpha-D-mannosidase activity, i.e. activity described in human Langerhans intraepidermal cells (Elleder, 1975). In the control group of tumors, with the exception of urticaria pigmentosa, enzyme activity was either at trace level or altogether absent. Acid alpha-D-mannosidase activity therefore appears to be the first biochemical feature common to both histiocytosis X and the Langerhans cells. The significance of the finding for the present theory of the histogenesis of the above tumors is discussed.
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PMID:alpha-D-mannosidase activity in histiocytosis X. 41 26

Three cases of congenital histiocytic disorders--generalized Langerhans cell histiocytosis, generalized juvenile xanthogranuloma and so-called congenital self-healing histiocytosis are compared using histiochemical, immunohistochemical and ultrastructural methods. The results showed a typical morphological pattern of Langerhans cell histiocytosis (S 100+, CD 1+, alpha-mannosidase +) with an unusual self-healing cutaneous phenomenon. The congenital self-healing histiocytosis showed a non-Langerhans cell immunophenotype (CD 14+, CD 1-, S 100-) and morphological appearance resembling the evolutive "early" stage of juvenile xanthogranuloma. A diffuse cellular positivity of alpha-mannosidase in juvenile xanthogranuloma and congenital self-healing histiocytosis differed from a typical perinuclear globular positivity of this enzyme in Langerhans cell histiocytosis. It is concluded that congenital self-healing histiocytosis may in some cases be of non-Langerhans cell type and under this term a clinically characteristic syndrome of histiocytic proliferation of Langerhans cells or tissue histiocytes may be included.
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PMID:Congenital histiocytosis. A heterogeneous group of diseases, one presenting as so-called congenital self-healing histiocytosis. 190 75

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

About 100 biopsy specimens, most of them from the skin, were used for comparisons between high alpha-D-mannosidase activity and the presence of Langerhans' cells identified according to the electronoptic picture. Agreement between the two was absolute so that alpha-D-mannosidase could be rated as a reliable index of Langerhans' cells not only in histiocytosis X but also in a number of non-tumour and other cancerous processes in the skin. -- Contact couples (apposition) of Langerhans' cells and lymphocytes were often found in as well as outside the epidermis in mycosis fungoides just as in skin allergoes of the contact dermatitis type (less so in experimental alergic dermatitis in the rat, guinea-pig, and rabbit caused by dinitrochlorobenzene). However, specific examinations of a number of electronograms from skin biopsies fairly often revealed such couples also in seborrhoeic keratosis, in basaliomas, and in other processes. -- Despite the above mentioned dispersion variance of the results obtained, Langerhans' cells may yet be found to represent a first link in a chain of immunity responses to exogenous antigens connected with ecotaxis and leading up to a picture of skin allergoses, dermatopathic reticulosis and mycosis fungoides.
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PMID:[Outline of the pathology of the Langerhans cell system]. 741 46