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一名3岁儿童复发性阵发性冷血红蛋白尿:病例报告

Recurrent paroxysmal cold haemoglobinuria in a 3-year-old child: a case report.

作者信息

Taylor C J C, Neilson J R, Chandra D, Ibrahim Z

机构信息

Medical Department, National Blood Service, Birmingham, UK.

出版信息

Transfus Med. 2003 Oct;13(5):319-21. doi: 10.1046/j.1365-3148.2003.00460.x.

Abstract

We present the case of a 3-year-old girl who presented on two separate occasions, eight months apart, with typical features of paroxysmal cold haemoglobinuria (PCH). On each occasion, she suffered malaise and fever with dark urine following an upper respiratory tract infection. She developed severe anaemia and required transfusion of red cells on both occasions, although each time she made a quick recovery. She was found to be direct antiglobulin test-positive with anti-C3d, and agglutination was observed on the blood film. A biphasic Donath-Landsteiner antibody was demonstrated. The specificity of the antibody was not identified, although it was not anti-P. As far as we are aware, recurrence of acute PCH in a child has not been previously reported in the literature.

摘要

我们报告了一名3岁女孩的病例,她在相隔8个月的两次不同就诊中表现出阵发性寒冷性血红蛋白尿(PCH)的典型特征。每次发病时,她在上呼吸道感染后都会出现不适、发热及深色尿。她出现了严重贫血,两次均需要输注红细胞,不过每次都恢复得很快。她的直接抗球蛋白试验显示抗C3d阳性,血涂片上观察到凝集现象。检测到了双相Donath-Landsteiner抗体。虽然该抗体不是抗-P,但未确定其特异性。据我们所知,儿童急性PCH复发此前在文献中尚无报道。

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