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儿童阵发性冷性血红蛋白尿:6例病例的治疗及不寻常临床表现回顾

Paroxysmal cold haemoglobinuria of childhood: a review of the management and unusual presenting features of six cases.

作者信息

Wynn R F, Stevens R F, Bolton-Maggs P H, Schwe K, Will A M

机构信息

Department of Paediatric Haematology, Royal Manchester Children's Hospital, Liverpool, UK.

出版信息

Clin Lab Haematol. 1998 Dec;20(6):373-5. doi: 10.1046/j.1365-2257.1998.00169.x.

DOI:10.1046/j.1365-2257.1998.00169.x
PMID:9951584
Abstract

We report six cases of children presenting with paroxysmal cold haemoglobinuria occurring in a 3-year period in the north-west of England. In all six cases the onset of the illness was dramatic and its duration brief. We note the prominent red cell agglutination was evident in the blood film, the absence of a prompt reticulocyte response and a negative classical direct Donath Landsteiner test in each of these cases. The Direct Coombs Test (DCT) was positive when anticomplement reagents were used but high titres of free autoantibody were not demonstrated in the serum.

摘要

我们报告了6例阵发性寒冷性血红蛋白尿患儿,这些病例发生在英格兰西北部3年期间。所有6例病例发病急骤,病程短暂。我们注意到,在这些病例的血片中均可见明显的红细胞凝集,网织红细胞反应未迅速出现,经典的直接Donath Landsteiner试验均为阴性。当使用抗补体试剂时,直接抗人球蛋白试验(DCT)呈阳性,但血清中未检测到高滴度的游离自身抗体。

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Paroxysmal cold haemoglobinuria of childhood: a review of the management and unusual presenting features of six cases.儿童阵发性冷性血红蛋白尿:6例病例的治疗及不寻常临床表现回顾
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引用本文的文献

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Paroxysmal cold hemoglobinuria successfully treated with complement inhibition.阵发性冷性血红蛋白尿症经补体抑制治疗后成功缓解。
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Diagnostic difficulties in a patient with paroxysmal cold haemoglobinuria and acute kidney injury.一名阵发性寒冷性血红蛋白尿症和急性肾损伤患者的诊断难题。
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