IgA 缺乏症中的伊文斯综合征。10年观察期内的发作性自身免疫性溶血性贫血和血小板减少症。

Evans' syndrome in IgA deficiency. Episodic autoimmune haemolytic anaemia and thrombocytopenia during a 10 years observation period.

作者信息

Hansen O P, Sørensen C H, Astrup L

出版信息

Scand J Haematol. 1982 Sep;29(3):265-70. doi: 10.1111/j.1600-0609.1982.tb00592.x.

DOI:10.1111/j.1600-0609.1982.tb00592.x

Abstract

A 26-year-old male with a 10-year history of complete selective IgA deficiency and recurrent autoimmune anaemia and thrombocytopenia (Evans syndrome) is presented. Both serum IgA and saliva secretory IgA were below the detection limit (less than 0.05 mg/l). No other features of autoimmunity were seen. The patient had a normal % of peripheral blood lymphocytes with surface IgM and IgG cells and normal in vitro lymphocyte transformation after stimulation with mitogens and antigens. The pleomorphic and randomly appearing immunologic features of selective IgA deficiency are emphasized by the present case.

摘要

本文报告了一名26岁男性，患有10年完全性选择性IgA缺乏症病史，伴有复发性自身免疫性贫血和血小板减少症（伊文斯综合征）。血清IgA和唾液分泌型IgA均低于检测限（小于0.05mg/L）。未发现其他自身免疫特征。该患者外周血中表面带有IgM和IgG的淋巴细胞百分比正常，用丝裂原和抗原刺激后体外淋巴细胞转化也正常。本病例强调了选择性IgA缺乏症多形性且随机出现的免疫学特征。