Mantadakis Elpis, Bezirgiannidou Zoe, Martinis George, Chatzimichael Athanassios
Department of Pediatrics, Democritus University of Thrace and University General District Hospital of Alexandroupolis, Thrace, Greece.
J Pediatr Hematol Oncol. 2011 Jan;33(1):40-2. doi: 10.1097/MPH.0b013e3181f46e77.
Hemolysis and hemoglobinuria after direct exposure to cold has rarely been reported in paroxysmal cold hemoglobinuria (PCH). The authors describe a 2.5-year-old boy with PCH (Donath-Landsteiner autoimmune hemolytic anemia), in whom 16 days after presentation, the hemoglobinuria and hemolysis recurred, when he was subjected to physical cooling, as a means to control fever associated with hospital-acquired croup. The hemolysis resolved with warmth, and administration of dexamethasone. PCH should be suspected in children with hemolytic anemia and positive direct antiglobulin test for complement. Avoidance of cold in the recovery period is imperative to prevent recurrences, whereas a short course of corticosteroids may be of benefit in suppressing the antibody production.
阵发性冷性血红蛋白尿(PCH)患者直接暴露于寒冷环境后出现溶血和血红蛋白尿的情况鲜有报道。作者描述了一名2.5岁患PCH(唐纳-兰德斯泰纳自身免疫性溶血性贫血)的男孩,其在就诊16天后,因采用物理降温控制与医院获得性哮吼相关的发热,再次出现血红蛋白尿和溶血。保暖及给予地塞米松后溶血症状缓解。对于溶血性贫血且直接抗球蛋白试验补体阳性的儿童,应怀疑患有PCH。恢复期必须避免寒冷以防止复发,而短期使用皮质类固醇可能有助于抑制抗体产生。