复发性多纳-兰德斯泰纳溶血性贫血：一例儿科病例报告。
Recurrent Donath-Landsteiner hemolytic anemia: a pediatric case report.
作者信息
Prince Sara D, Winestone Lena E, Nance Sandra J, Friedman David F
机构信息
Nemours Alfred I. DuPont Hospital for Children, Wilmington, Delaware.
Division of Pediatric Hematology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
出版信息
Transfusion. 2017 Jun;57(6):1401-1406. doi: 10.1111/trf.14032. Epub 2017 Mar 31.
BACKGROUND
Paroxysmal cold hemoglobinuria (PCH) is a form of autoimmune hemolytic anemia caused by the Donath-Landsteiner antibody (D-L antibody). In children, this is typically a transient immune-mediated hemolysis that follows a viral illness and does not recur. Recurrent acute or chronic PCH due to D-L antibody is very rare.
CASE REPORT
We have reported a unique case of recurrent PCH in a 5-year-old boy with two acute episodes of hemolysis separated by 21 months of hematologic remission. Each episode was severe requiring red blood cell transfusions, intravenous methylprednisolone, and intravenous immunoglobulin during the second episode. Testing identified recurrence of the D-L antibody with the classic anti-P biphasic hemolysis.
CONCLUSION
This demonstrates that PCH can be a recurrent disease in the pediatric population (in the absence of syphilis) with the classical D-L antibody.
背景
阵发性冷性血红蛋白尿（PCH）是一种由多纳-兰德斯泰纳抗体（D-L抗体）引起的自身免疫性溶血性贫血。在儿童中，这通常是一种短暂的免疫介导性溶血，发生在病毒感染后，不会复发。由D-L抗体引起的复发性急性或慢性PCH非常罕见。
病例报告
我们报告了一例独特的复发性PCH病例，患者为一名5岁男孩，有两次急性溶血发作，两次发作间隔21个月的血液学缓解期。每次发作都很严重，第二次发作时需要输注红细胞、静脉注射甲泼尼龙和静脉注射免疫球蛋白。检测发现D-L抗体复发，并伴有典型的抗-P双相溶血。
结论
这表明在儿科人群中（无梅毒），PCH可成为由经典D-L抗体引起的复发性疾病。
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