阵发性寒冷性血红蛋白尿。儿童中最常见的急性自身免疫性溶血性贫血？

Paroxysmal cold haemoglobinuria. The most frequent acute autoimmune haemolytic anaemia in children?

作者信息

Nordhagen R, Stensvold K, Winsnes A, Skyberg D, Støren A

出版信息

Acta Paediatr Scand. 1984 Mar;73(2):258-62. doi: 10.1111/j.1651-2227.1984.tb09939.x.

DOI:10.1111/j.1651-2227.1984.tb09939.x

Abstract

Paroxysmal cold haemoglobinuria (PCH) is a disease which today is met mainly in its acute form in children. The diagnosis is revealed by a careful serological examination. Case reports of four children with PCH are given in this paper. They all showed typical clinical pictures of PCH, and displayed the expected serological findings, including a bithermic autoantibody (haemolysin), the Donath-Landsteiner antibody. Children with PCH often require immediate transfusion therapy, but certain precautions should be taken regarding transfusion policy in these patients. When the acute phase is overcome, the prognosis is excellent.

摘要

阵发性寒冷性血红蛋白尿（PCH）是一种如今主要在儿童中以急性形式出现的疾病。通过仔细的血清学检查可做出诊断。本文给出了4例患有PCH的儿童病例报告。他们均表现出PCH的典型临床症状，并呈现出预期的血清学检查结果，包括双相自身抗体（溶血素），即唐纳-兰斯泰纳抗体。患有PCH的儿童常需要立即进行输血治疗，但对于这些患者的输血策略应采取某些预防措施。急性期过后，预后良好。