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[成人型特发性阵发性冷血红蛋白尿症]

[Adult type of idiopathic paroxysmal cold hemoglobinuria].

作者信息

Fukuda M, Furuya H, Notsu K, Takagi C, Suyama Y, Ishikura H, Kato Y

机构信息

Department of Medicine, Shimane Medical University.

出版信息

Rinsho Ketsueki. 1991 Nov;32(11):1498-502.

PMID:1758060
Abstract

A 78-year-old female suffered from idiopathic paroxysmal cold hemoglobinuria (PCH). Her symptom occurred immediately after she worked outside in the cold early morning. This case is characterized by the eldest case with non-syphilitic PCH reported in Japan and by the most advanced anemia (Hb 4.2 g/dl) possibly due to prolonged hemolysis after cold exposure. Analysis of the serum revealed positive Donath-Landsteiner antibody of IgG type, which could react to hemolysis not only below 15 degrees C but also at 15-20 degrees C in vitro.

摘要

一名78岁女性患有特发性阵发性寒冷性血红蛋白尿(PCH)。她在清晨寒冷天气外出工作后立即出现症状。该病例的特点是,这是日本报道的年龄最大的非梅毒型PCH病例,且可能由于寒冷暴露后长时间溶血导致贫血最为严重(血红蛋白4.2 g/dl)。血清分析显示IgG型的Donath-Landsteiner抗体呈阳性,该抗体不仅在体外15摄氏度以下,而且在15至20摄氏度时均可引发溶血反应。

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