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[儿童阵发性寒冷性血红蛋白尿症合并急性溶血]

[Paroxysmal cold hemoglobinuria complicated with acute hemolysis in a child].

作者信息

Hirota Y, Saito H, Yamada K, Ishikawa A

出版信息

Rinsho Ketsueki. 1989 Apr;30(4):466-70.

PMID:2769969
Abstract

An 8 year-old girl with paroxsmal cold hemoglogunuria (PCH) is reported. She was referred to our clinic because of acute anemia, and found to have PCH after clinical and laboratory examination. Anti-P1 antibody and Donath-Landsteiner (D-L) antibody were detected. In Coombs' test with variable incubation temperatures, positive IgG on her erythrocytes changed to negative as temperature of incubation was elevated. Also activation of complement was detected on the surface of her erythrocytes. Clinical improvement was obtained by blood transfusion, and positive Coomb's test and D-L test turned to negative.

摘要

报告了一名8岁患阵发性寒冷性血红蛋白尿(PCH)的女孩。她因急性贫血转诊至我们的诊所,经临床和实验室检查后被诊断为PCH。检测到抗P1抗体和唐纳-兰斯泰纳(D-L)抗体。在不同孵育温度的库姆斯试验中,随着孵育温度升高,其红细胞上阳性的IgG变为阴性。同时在其红细胞表面检测到补体激活。通过输血临床症状改善,库姆斯试验阳性和D-L试验转为阴性。

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Rinsho Ketsueki. 1989 Apr;30(4):466-70.
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