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华氏巨球蛋白血症并发自身免疫性溶血性贫血、脑膜刺激征和股骨溶解。

Macroglobulinemia Waldenström complicated by autoimmune hemolytic anemia, meningeal sign and femoral lysis.

作者信息

Tanaka T, Ueda N, Fujita M, Oka A, Tanaka S, Sezaki T, Yamamoto K, Shiomi H

出版信息

Acta Pathol Jpn. 1979 Sep;29(5):777-89. doi: 10.1111/j.1440-1827.1979.tb00943.x.

Abstract

A necropsied case of macroglobulinemia Waldenström with unusual complications was presented; the patient was found to have autoimmune hemolytic anemia, first. During the course of its treatment, he developed monoclonal immunoglobulin M in serum together with lytic osseous lesion in both femurs, and died of meningeal involvement by tumor cells. Clinically, he had hepatosplenomegaly and generalized lymphadenopathy. The macroglobulin was kappa type of light chain, ultracentrifugation showed sharp spike of 21S in 18.6%, and myelogram revealed increased lymphocytoid series of 29.6%. It was proved that autoantibody causing autoimmune hemolytic anemia was composed of IgG and macroglobulin itself had no activity as an anti-red cell antibody. An emphasis was made that autoimmune hemolytic anemia followed by macroglobulinemia is based on the state of immunodeficiency of this patient.

摘要

本文报告了一例伴有罕见并发症的华氏巨球蛋白血症尸检病例;该患者首先被发现患有自身免疫性溶血性贫血。在治疗过程中,他血清中出现单克隆免疫球蛋白M,同时双侧股骨出现溶骨性病变,最终死于肿瘤细胞脑膜浸润。临床上,他有肝脾肿大和全身淋巴结肿大。巨球蛋白为κ型轻链,超速离心显示18.6%出现21S的尖锐峰,骨髓象显示淋巴细胞样系列增加29.6%。已证实导致自身免疫性溶血性贫血的自身抗体由IgG组成,而巨球蛋白本身作为抗红细胞抗体无活性。强调自身免疫性溶血性贫血继发巨球蛋白血症是基于该患者的免疫缺陷状态。

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