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具有IgM副蛋白的原发性单克隆丙种球蛋白病,该副蛋白是一种具有冷凝集素和EDTA依赖性血小板抗体特性的冷球蛋白。

Essential monoclonal gammopathy with an IgM paraprotein that is a cryoglobulin with cold agglutinin and EDTA-dependent platelet antibody properties.

作者信息

Pujol M, Ribera J M, Jimenez C, Ribera A, Abad E, Feliu E

机构信息

Servicio de Hematología y Hemoterapia del Hospital Universitario Germans Trias y Pujol, Badalona, Spain.

出版信息

Br J Haematol. 1998 Mar;100(3):603-4. doi: 10.1046/j.1365-2141.1998.00586.x.

Abstract

A patient with apparent anaemia and thrombocytopenia caused by a monoclonal paraprotein is described. The patient's serum contained a monoclonal IgM kappa, a cryoglobulin and a cold agglutinin. The cryoglobulin, similar to the serum paraprotein, was a monoclonal IgM kappa. Serum was studied to determine the relationship of the cryoglobulin with the cold agglutinin. The cryoglobulin and cold agglutinin were found to be the same paraprotein. Moreover, with absorption and elution techniques the reactivity of the autoantibody with both erythrocytes and platelets was demonstrated. Reports of cryoprecipitable cold agglutinins are rare and therefore this case is exceptional given that the IgM kappa paraprotein was found to be a cold agglutinin which was also reactive with platelets.

摘要

本文描述了一名由单克隆副蛋白引起明显贫血和血小板减少的患者。患者血清中含有单克隆IgM κ、冷球蛋白和冷凝集素。该冷球蛋白与血清副蛋白相似,为单克隆IgM κ。对血清进行研究以确定冷球蛋白与冷凝集素之间的关系。发现冷球蛋白和冷凝集素为同一副蛋白。此外,通过吸收和洗脱技术证实了自身抗体与红细胞和血小板均具有反应性。关于可冷沉淀冷凝集素的报道很少,因此该病例较为特殊,因为发现IgM κ副蛋白是一种冷凝集素,且对血小板也有反应性。

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