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伴有IgM λ型冷沉淀性冷凝集素的低温性坏疽

Cryopathic gangrene with an IgM lambda cryoprecipitating cold agglutinin.

作者信息

Kuenn J W, Weber R, Teague P O, Keitt A S

出版信息

Cancer. 1978 Oct;42(4):1826-33. doi: 10.1002/1097-0142(197810)42:4<1826::aid-cncr2820420422>3.0.co;2-x.

Abstract

Immunochemical and serologic studies of cold agglutinis in patients with chronic cold agglutinin disease (CCAD) have shown the almost exclusive occurrence of IgM kappa antibodies with specificity for the I antigen of red cells. An unusual subgroup of patients has been delineated in which the cryoprotein is IgM lambda, frequently lacks I specificity and often cryoprecipitates. Studies of such a protein from a patient with an unusual array of immunoproliferative disorders including Grave's disease with exophthalmos and Waldenstrom's macroglobulinemia indicate that the cryoprecipitating and cold agglutinating properties probably derive from the sam protein. The occurrence of this type of antibody should suggest the presence of a more aggressive lymphoproliferative disorder than simple CCAD.

摘要

对慢性冷凝集素病(CCAD)患者冷凝集素的免疫化学和血清学研究表明,几乎仅出现对红细胞I抗原具有特异性的IgM κ抗体。已划定了一个不寻常的患者亚组,其中冷球蛋白是IgM λ,通常缺乏I特异性且常发生冷沉淀。对一名患有包括突眼性格雷夫斯病和瓦尔登斯特伦巨球蛋白血症等一系列不寻常免疫增殖性疾病患者的此类蛋白进行研究表明,冷沉淀和冷凝集特性可能源自同一蛋白。这类抗体的出现应提示存在比单纯CCAD更具侵袭性的淋巴增殖性疾病。

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