Division of Transfusion Medicine, Department of Pathology, The Johns Hopkins Hospital, Baltimore, Maryland, USA.
Pediatr Blood Cancer. 2012 Nov;59(5):953-5. doi: 10.1002/pbc.24185. Epub 2012 May 2.
In children, paroxysmal cold hemoglobinuria (PCH) is generally considered an acute self-limited autoimmune hemolytic anemia caused by an IgG biphasic auto-anti-P antibody identified by the Donath-Landsteiner (D-L) test. We report a case of a 5-year-old female with a chronic hemolytic anemia. The etiology of the hemolysis appears to be an unusual D-L positive, IgM antibody with specificity for the I antigen. The clinical course is described and a discussion of PCH and the D-L antibody is presented. We also discuss intravenous immunoglobulin infusions as a therapy for children with this form of severe chronic autoimmune hemolytic anemia.
在儿童中，阵发性冷性血红蛋白尿症（PCH）通常被认为是一种由 IgG 双相自身抗-P 抗体引起的急性自限性自身免疫性溶血性贫血，该抗体可通过 Donath-Landsteiner（D-L）试验来识别。我们报告了一例 5 岁女性慢性溶血性贫血病例。溶血的病因似乎是一种不常见的 D-L 阳性 IgM 抗体，其特异性为 I 抗原。本文描述了该病例的临床经过，并讨论了 PCH 和 D-L 抗体。我们还讨论了静脉注射免疫球蛋白输注作为治疗这种严重慢性自身免疫性溶血性贫血的方法。
