表现为复发性溶血尿毒综合征的阵发性夜间血红蛋白尿

Paroxysmal nocturnal hemoglobinuria presenting as recurrent hemolytic uremic syndrome.

作者信息

Kletzel M, Arnold W C, Berry D H

出版信息

Clin Pediatr (Phila). 1987 Jun;26(6):319-20. doi: 10.1177/000992288702600613.

Abstract

Paroxysmal nocturnal hemoglobinuria (PNH) may present with acute anemia, thrombocytopenia and, if hemoglobin nephropathy or dehydration is present, azotemia. Thus PNH may be confused with the hemolytic uremic syndrome (HUS). Recurrent episodes, though common in PNH, are unusual in HUS. A positive acid hemolysis test can be used to differentiate between the two diseases.

摘要

阵发性夜间血红蛋白尿（PNH）可能表现为急性贫血、血小板减少，并且如果存在血红蛋白肾病或脱水，则可能出现氮质血症。因此，PNH可能会与溶血性尿毒症综合征（HUS）相混淆。虽然反复发作在PNH中很常见，但在HUS中并不常见。酸溶血试验阳性可用于区分这两种疾病。