单词 | letterer-siwe disease |
释义 | Letterer-Siwe diseaseLetterer-Siwe disease[′led·ə·rər ′zē·və di‚zēz]Letterer-Siwe diseaseLetterer-Siwe disease[let´er-er si´we]Let·te·rer-Si·we dis·ease(let'ĕr-ĕr sē'vĕ),Letterer-Siwe disease(lĕt′ər-ər-sī′wē, -sē′və)Letterer-Siwe diseaseA form of Langerhans' cell histiocytosis or histiocytosis X, with an onset in infancy of fever, eczematoid rash, lymphadenopathy, hepatosplenomegaly, anemia, death. See Histiocytosis, Langerhans' cell histiocytosis.Let·te·rer-Si·we dis·ease(let-ĕr'er sē'vē di-zēz')Synonym(s): nonlipid histiocytosis. Letterer-Siwe diseaseA fatal disease of infants in which there is fever, generalized lymph node, liver and spleen enlargement, an eczematous skin rash and anaemia. The condition is brought about by an enormous increase in the number of antigen-presenting cells of the immune system known as Langerhans cells. These produce grossly excessive CYTOKINES resulting in great proliferation. The mortality varies but may be as high as 50 per cent. Experts argue as to whether or not the condition is a form of cancer. The preferred name for the condition is now Langerhans cell histiocytosis. (Erich Letterer, German pathologist, b. 1895, and Sture August Siwe, Swedish paediatrician, b. 1897).Letterer,Erich, German pathologist, 1895-1932.Siwe,Sture August, Swedish pediatrician, 1897-1966.Let·te·rer-Si·we dis·ease(let'er-er-sē'vē di-zēz')Synonym(s): nonlipid histiocytosis. See LESD |
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