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[华氏巨球蛋白血症中的血小板减少症与IgM副蛋白的特异性抗血小板特性]

[Thrombocytopenia in Waldenstrom's disease with specific antithrombocytic properties of the IgM paraprotein].

作者信息

Flury W, Riesen W, Morell A, Keller H

出版信息

Schweiz Med Wochenschr. 1975 Sep 27;105(39):1241-5.

PMID:809839
Abstract

The serum of a patient suffering from thrombocytopenia associated with Waldenström's macroglobulinemia was found to contain an IgM paraprotein with unusual characteristics. Following isolation of this IgM paraprotein, dilutions up to 0.01 mg/ml caused immunofluorescence of thrombocytes from man, sheep, rabbit, and horse. In control studies with isolated IgM paraprotein from another non-thrombocytopenic patient with Waldenström's disease, positive immunofluorescence could be elicited only when a 1000-fold greater concentration of the paraprotein was used. The IgM paraprotein from our thrombocytopenic patient and that from the control patient showed comparable differences in immunofluorescence of thrombocytes and megacaryocytes when bone marrow smears were used for incubation. Further studies following papain cleavage of the IgM paraprotein with antithrombocytic activity showed that the combining activity was located in the Fab fragment. This observation characterizes the IgM paraprotein from our patient as an antibody directed to a substance in human and animal thrombocytes. Paraproteins with antibody-like activity for thrombocytes have not been identified previously; the present observation suggests that this mechanism should be considered a possible cause of thrombocytopenia associated with macroglobulinemia.

摘要

发现一名患有与华氏巨球蛋白血症相关的血小板减少症患者的血清中含有一种具有异常特征的IgM副蛋白。分离出这种IgM副蛋白后,稀释至0.01mg/ml仍能使来自人、绵羊、兔子和马的血小板产生免疫荧光。在对另一名患有华氏病的非血小板减少症患者分离出的IgM副蛋白进行对照研究时,只有使用浓度高1000倍的副蛋白才能引发阳性免疫荧光。当使用骨髓涂片进行孵育时,来自血小板减少症患者的IgM副蛋白和对照患者的IgM副蛋白在血小板和巨核细胞的免疫荧光方面表现出类似差异。在用木瓜蛋白酶裂解具有抗血小板活性的IgM副蛋白后进行的进一步研究表明,结合活性位于Fab片段中。这一观察结果表明,我们患者的IgM副蛋白是一种针对人和动物血小板中某种物质的抗体。此前尚未发现具有类似血小板抗体活性的副蛋白;目前的观察结果表明,这种机制应被视为与巨球蛋白血症相关的血小板减少症的一个可能原因。

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