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)

Using a monoclonal antibody specific to the Lewis X antigen (anti-Lex), the authors studied 103 cases of Hodgkin's disease (HD) in comparison with 57 cases of non-Hodgkin's lymphoma (NHL); three cases of granulocytic sarcoma (GS); two cases of malignant histiocytosis (MH); one case of monoblastic leukemia (ML); one case of interdigitating reticulum cell sarcoma (IRCS); six cases of histiocytosis X (HX); one case of reticulohistiocytoma (RH); 44 various reactive conditions of the lymph node (LN). Reed-Sternberg and related (R-S) cells stained selectively in 80 of 92 cases of HD (87.0%), excluding 11 cases of lymphocyte predominance type. The stain was better in B-5-fixed specimens than in formalin-fixed specimens, showing a dense deposit of reaction products at a paranuclear site and on the cell surface. The staining results were compared with those of Leu-M1 and found to be superior both qualitatively and quantitatively (detection rate of R-S cells: 87.0% versus 68.5% of Leu-M1). Granulocytes, rare epithelioid histiocytes, and some endothelial and/or erythrocytes also stained with anti-Lex. The stain had positive results in three cases of GS showing a diffuse cytoplasmic staining pattern. Of NHL, two of 29 peripheral T-cell lymphomas stained to show rare paranuclear deposits without cell surface staining. The stain had negative results in MH, ML, IRCS, HX, and RH. Of 45 reactive LN, minute subcapsular collections of Lewis X+, altered-appearing Langerhans'-like cells, were observed in all ten LN from human immunodeficiency virus (HIV)-associated persistent generalized lymphadenopathy (PGL). The stain had negative results in all other various reactive conditions of LN. In conclusion, Lewis X staining is useful as a marker for R-S cells in paraffin sections with staining results superior to those of Leu-M1. Lewis X staining also detects subcapsular clustering of altered-appearing Langerhans'-like cells in PGL, which has not been described previously and warrants additional study.
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PMID:The Lewis X antigen. A new paraffin section marker for Reed-Sternberg cells. 170 18

The histiocytoses, whether reactive or neoplastic, can be related histologically and immunophenotypically to their normal counterparts within the histiocytic system. This system has two subsets: The dendritic (antigen-presenting) cells and the phagocytic histiocytic (antigen-processing) cells. Dermatopathic lymphadenitis and Langerhans cell histiocytosis (histiocytosis X) are reactive proliferative disorders of dendritic cells. Malignancies of dendritic cells exist, but they are very rare. Benign proliferations of phagocytic histiocytes include the hemophagocytic syndromes, both familial and reactive, as well as sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) and histiocytic necrotizing lymphadenitis (Kikuchi's disease). Neoplasms of phagocytic histiocytes include acute monocytic leukemia and the very rare entities, malignant histiocytosis and true histiocytic lymphoma. The latter must be distinguished from sinusoidal, large cell anaplastic lymphomas.
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PMID:The histiocytoses: clinical presentation and differential diagnosis. 215 Mar 25

The term histiocyte refers to cells of either the macrophage or Langerhans cell lineages. The histiocytic disorders are characterized by the proliferation of cells of these lineages. With recent advances in knowledge of the developmental biology of histiocytic cells, it is now possible to formulate a reasonable catalogue of histiocytic diseases based on ultra-structural and phenotypic markers of cellular origins and molecular or chromosomal markers of malignancy. The catalogue includes the following groups of diseases. Nonmalignant reactive macrophage disorders include (1) macrophage storage diseases, (2) several benign proliferative macrophage disorders that predominantly involve skin and bone, and (3) several hemophagocytic syndromes that vary from indolent and benign to fulminant and fatal. In some of the latter disorders, viruses have been identified as the inciting stimulus. The malignant macrophage disorders include (1) acute monocytic leukemia and (2) chronic myelomonocytic leukemia. A rare disorder that gave rise to a permanent cell line with an anomaly of chromosomal segment 5q35 may also be an example of a histiocytic malignancy. The existence of a separate category of true histiocytic lymphoma of macrophage type is uncertain. Reactive Langerhans cell disorders include (1) congenital self-healing histiocytosis, (2) the many variants of eosinophilic granuloma, and (3) a related disorder designated as relapsing Langerhans cell histiocytosis that is characterized by a relapsing course and infiltration of bone and soft tissues by Langerhans cells. Presumptively neoplastic diseases of Langerhans and dendritic cells include (1) progressive Langerhans cell histiocytosis, a disease with prominent involvement of blood and BM as well as skin and viscera; (2) Langerhans cell lymphoma, and (3) dendritic cell lymphoma. However, clonality as a marker of malignancy has not been proven in these disorders.
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PMID:Histiocytes and histiocytosis. 771 2

Retrospective analysis of childhood histiocytoses treated at the Department of Pediatrics, Ramathibodi Hospital from May 1970 till June 30, 1992 to verify the prevalence according to the recent classification, course and prognosis was conducted. Among 120 cases, 54 were Class I or Langerhan Cell Histiocytosis (LCH, previously called Histiocytosis-X). Nineteen cases were Class II: Infection-Associated Hemophagocytic Syndrome (IAHS) and Sinus Histiocytosis. Forty-seven cases were class III which included acute monocytic leukemia, juvenile CML; malignant histiocytosis, HMR, and histiocytic lymphoma. Excellent prognosis (cure) was seen in all cases of Hand-Schuller Christian Disease, eosinophilic granuloma, sinus histiocytosis with massive lymphadenopathy and many cases of Class II (except IAHS). The worse prognosis (100% mortality rate) was seen in HMR and juvenile CML. The intermediate prognosis (50%, 54%, 58.8% and 66.7% mortality rate) was seen in MH, HL, LSD and IAHS respectively. To differentiate between IAHS and MH/HMR, the prominent bone marrow findings in 12 cases of IAHS revealed that every case showed prominent hemophagocytosis by the promono-histiocytes/histiocytes, the maximal total erythroblasts (TE) were only 7.5 per cent except for 3 cases; in which one case with agranulocytosis from co-trimoxazole had 84 per cent erythroid cells, one case with prior co-trimoxazole treatment had 37 per cent TE, the last one had 40 per cent TE with massive GI bleeding while in the recovery stage of DHF. All 5 cases of HMR had prominent hemophagocytosis and increased TE (> 22%).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Histiocytoses in children: analysis of 120 cases and the bone marrow findings in infection-induced hemophagocytic syndrome vs malignant histiocytosis. 782 9

Sclerosing cholangitis in childhood is a heterogeneous condition, which has different aetiologies. Sclerosing cholangitis may be inherited and diagnosed in the neonatal period (neonatal sclerosing cholangitis); it may present later with features of autoimmunity (autoimmune sclerosing cholangitis); or it may be associated with a variety of disorders, including Langerhans cell histiocytosis, immunodeficiency, psoriasis, cystic fibrosis, reticulum cell sarcoma and sickle cell anaemia. In contrast to the experience in adult patients, sclerosing cholangitis occurring as an individual disease (primary sclerosing cholangitis) is rare. The initiating events and possible pathogenic mechanisms differ in the various forms of sclerosing cholangitis and are still obscure. Treatment and prognosis depend on the type of sclerosing cholangitis present.
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PMID:Sclerosing cholangitis in the paediatric patient. 1149 76

Dendritic cell neoplasms of the World Health Organization classification comprise Langerhans cell histiocytosis, Langerhans cell sarcoma, interdigitating dendritic cell sarcoma, follicular dendritic cell sarcoma, and dendritic cell sarcoma, not otherwise specified. Several studies based on immunohistochemical and ultrastructural analysis tried to further clarify the origin of these neoplasms which are thought to derive from mesenchymal or bone marrow precursors. Lymphatic vessel endothelium hyaluronan receptor-1 (LYVE-1) was recently described as a marker for lymphatic endothelium which is expressed on normal liver blood sinusoid lining cells, spleen endothelium, activated tissue macrophages, blood vessels in the lung, endothelial cells of lymphatic sinuses, and in fibroblastic reticular cells in lymph nodes. We present a case of LYVE-1-positive reticulum cell neoplasm in an axillary lymph node. To the best of our knowledge, there has been no report about LYVE-1 expression in histiocytic or dendritic cell neoplasms so far. Due to the assumed specificity of this antibody, we propose designation of this reticulum cell sarcoma as lymphatic sinus lining cell sarcoma which might finally represent another subtype of reticulum cell sarcomas.
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PMID:Sarcoma of follicular dendritic cells with features of sinus lining cells--a new subtype of reticulum cell sarcoma? 1838 53