Delayed hemolytic transfusion reaction in children with sickle cell disease
Delayed hemolytic transfusion reaction in children with sickle cell disease
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DOI：
10.3324/haematol.2010.038307
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发表时间：
2011-06-01
期刊：
影响因子：
--
通讯作者：
Noizat-Pirenne, France
中科院分区：
文献类型：
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作者：
de Montalembert, Mariane;Dumont, Marie-Dominique;Noizat-Pirenne, France
背景输血是治疗镰状细胞病的基石，但可能是由于欧洲血统的献血者和非洲血统的患者在红细胞抗原上的差异，所以输血反应的风险很高。患者可能会出现溶血性输血反应，延迟几天到两周，表现为急性溶血(血红蛋白尿、黄疸和脸色苍白)，症状提示严重的血管闭塞危象(疼痛、发热和急性胸部综合征)，以及严重贫血，通常伴有网织红细胞减少。本病例系列研究旨在描述该综合征的主要特征，讨论其病理生理学，并提出处理策略。设计与方法我们在法国内克医院的数据库中确定了2006-2009年间的8例儿童迟发性溶血性输血反应病例。所有患者都接受了ABO、RH和KEL系统相容的交叉配型红细胞单位。我们查阅了计算机化输血数据库中的病历。所有患者都住进了重症监护室。当网织红细胞数低于150×10(9)/L时，逐步采用静脉注射免疫球蛋白和促红细胞生成素α的策略，急性期除非绝对必要，否则不再输血。所有患者都有严重的骨痛；除了一人外，所有人都有高烧。5例患者血红蛋白低于4g/dL，3例网织红细胞减少。5例患者未发现新抗体，1例为弱反应性抗体。仅2例患者出现新的同种异体抗体，可能与迟发性溶血反应有关。结论迟发性溶血反应的首发症状复杂，与镰状细胞病的其他并发症相似。在我们的大多数病例中，没有发现新的抗体，这突显了这种综合征病理生理学的复杂性。
BackgroundTransfusion is a cornerstone of the management of sickle cell disease but carries a high risk of hemolytic transfusion reaction, probably because of differences in erythrocyte antigens between blood donors of European descent and patients of African descent. Patients may experience hemolytic transfusion reactions that are delayed by from a few days to two weeks and manifest as acute hemolysis (hemoglobinuria, jaundice, and pallor), symptoms suggesting severe vaso-occlusive crisis (pain, fever, and acute chest syndrome), and profound anemia, often with reticulocytopenia. This case-series study aims to describe the main characteristics of this syndrome, to discuss its pathophysiology, and to propose a management strategy.Design and MethodsWe identified 8 pediatric cases of delayed hemolytic transfusion reactions between 2006 and 2009 in the database of the Necker Hospital, France. All patients had received cross-matched red cell units compatible in the ABO, RH, and KEL systems. We reviewed the medical charts in the computerized blood transfusion databases. All patients were admitted to the intensive care unit. We progressively adopted the following strategy: intravenous immunoglobulins, and darbopoietin alpha when the reticulocyte count was below 150x10(9)/L, without further blood transfusion during the acute episode unless absolutely necessary.ResultsThe median time between the transfusion and the diagnosis of delayed hemolytic transfusion reaction was six days. All patients had severe bone pain; all but one had a high-grade fever. Five patients had hemoglobin levels less than than 4g/dL and 3 had reticulocytopenia. In 5 patients, no new antibody was found; one patient had weakly reactive antibodies. Only 2 patients had new allo-antibodies possibly responsible for the delayed hemolytic reaction.ConclusionsThe initial symptoms of delayed hemolytic transfusion reaction were complex and mimicked other complications of sickle cell disease. In most of our cases, no new antibody was identified, which underlines the complexity of the pathophysiology of this syndrome.