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慢性冷凝集素病中的双克隆丙种球蛋白病

Diclonal gammopathy in chronic cold agglutinin disease.

作者信息

Fingerle R E, Check I J

出版信息

Am J Clin Pathol. 1982 Dec;78(6):867-70. doi: 10.1093/ajcp/78.6.867.

DOI:10.1093/ajcp/78.6.867
PMID:6816062
Abstract

Serum from a patient with chronic cold agglutinin disease (CCAD) contained two paraproteins identified as IgM kappa and IgM lambda by immunoelectrophoresis and confirmed by immunofixation on high resolution agarose gel electrophoresis. The IgM kappa band was completely removed by incubation of the serum at 4 degrees with ficin-treated O Rh-positive red blood cells, and partially removed by incubation with cord cells, suggesting that it reacted with I antigen. The IgM lambda band was unaffected by these absorptions. Previous investigators have shown CCAD to be a monoclonal gammopathy, usually IgM kappa. The presence of both IgM kappa and IgM lambda paraproteins in this patient with the IgM kappa showing cold agglutinin activity is unique. The patient has a lymphoplasmacytic infiltrate in the bone marrow. These data suggest that CCAD may be part of a spectrum of disorders in which paraproteins are produced and are stimulated by factors unrelated to the target red blood cell antigens.

摘要

一名慢性冷凝集素病(CCAD)患者的血清中含有两种副蛋白,经免疫电泳鉴定为IgM κ和IgM λ,并通过高分辨率琼脂糖凝胶电泳上的免疫固定法得到确认。将血清与经ficin处理的O型Rh阳性红细胞在4℃孵育后,IgM κ条带完全消失,与脐血红细胞孵育后部分消失,提示其与I抗原发生反应。IgM λ条带不受这些吸收作用的影响。既往研究表明CCAD是一种单克隆丙种球蛋白病,通常为IgM κ型。该患者同时存在IgM κ和IgM λ副蛋白,且IgM κ具有冷凝集素活性,这种情况很独特。该患者骨髓中有淋巴细胞浆细胞浸润。这些数据表明,CCAD可能是一系列疾病的一部分,在这些疾病中会产生副蛋白,并受到与靶红细胞抗原无关的因素刺激。

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Diclonal gammopathy in chronic cold agglutinin disease.慢性冷凝集素病中的双克隆丙种球蛋白病
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2
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Waldenström's macroglobulinemia with an IgM paraprotein that is both a cold agglutinin and a cryoglobulin and has a suppressive effect on progenitor cell growth.华氏巨球蛋白血症,其免疫球蛋白M型副蛋白既是冷凝集素又是冷球蛋白,且对祖细胞生长具有抑制作用。
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[Progression from Crow-Fukase syndrome with double gammopathy (IgM-kappa, IgG-lambda) to primary macroglobulinemia].[从伴有双克隆丙种球蛋白病(IgM-κ、IgG-λ)的克劳-深泽综合征进展为原发性巨球蛋白血症]
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