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一例罕见的华氏巨球蛋白血症病例,血清和尿液中存在IgG半分子。

An unusual case of Waldenström macroglobulinemia with half molecules of IgG in serum and urine.

作者信息

Gallango M L, Suinaga R, Ramírez M

出版信息

Blut. 1984 Feb;48(2):91-7. doi: 10.1007/BF00320035.

Abstract

Immunochemical studies are described in an unusual case of Waldenström's macroglobulinemia. Two monoclonal Igs (whole IgG1/kappa and IgG1/kappa half molecules) occurred in the serum in addition to the IgM monoclonal protein. Protein electrophoresis of the serum showed a monoclonal component in the gamma region, and the immunoelectrophoresis allowed detection of a monoclonal IgM/kappa and another abnormality represented by a double precipitin line in serum and urine, observed when antiserum anti IgG was used. The abnormal proteins were purified and further analyzed. The IgG-related proteins were whole four chains IgG monoclonal molecules, 1/2 IgG monoclonal molecules, composed of one heavy and one light chain, and residual polyclonal IgG. The half molecules were antigenically deficient with respect to normal IgG. The idiotypic analysis showed that the three monoclonal proteins shared idiotypic determinants. This patient had clinical and morphological findings of Waldenström's macroglobulinemia and, as observed in other cases, the formation of half molecules was not associated with a distinct clinical syndrome.

摘要

本文描述了一例罕见的华氏巨球蛋白血症患者的免疫化学研究情况。除了IgM单克隆蛋白外,血清中还出现了两种单克隆免疫球蛋白(完整的IgG1/κ和IgG1/κ半分子)。血清蛋白电泳显示γ区有一个单克隆成分,免疫电泳可检测到单克隆IgM/κ以及血清和尿液中由双沉淀线代表的另一种异常情况,该情况在用抗IgG抗血清时观察到。对异常蛋白进行了纯化并进一步分析。与IgG相关的蛋白有完整的四条链IgG单克隆分子、由一条重链和一条轻链组成的1/2 IgG单克隆分子以及残留的多克隆IgG。半分子相对于正常IgG在抗原性上有缺陷。独特型分析表明这三种单克隆蛋白具有共同的独特型决定簇。该患者具有华氏巨球蛋白血症的临床和形态学表现,并且如同在其他病例中观察到的那样,半分子的形成与一种独特的临床综合征无关。

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本文引用的文献

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Antigen-antibody reactions in gels.凝胶中的抗原-抗体反应
Acta Pathol Microbiol Scand. 1949;26(4):507-15. doi: 10.1111/j.1699-0463.1949.tb00751.x.
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[A micro-method of immuno-electrophoresis].[免疫电泳的微量方法]
Int Arch Allergy Appl Immunol. 1955;7(2):103-10.
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Br Med J. 1971 Apr 10;2(5753):67-72. doi: 10.1136/bmj.2.5753.67.
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Human myeloma IgA half-molecules.人骨髓瘤IgA半分子
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