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首页> 外文期刊>Pediatric transplantation. >Platelet transfusion refractoriness in highly immunized beta thalassemia children undergoing stem cell transplantation.
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Platelet transfusion refractoriness in highly immunized beta thalassemia children undergoing stem cell transplantation.

机译:接受干细胞移植的高度免疫的β地中海贫血儿童的血小板输注不全。

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摘要

Immune-mediated refractoriness to platelet transfusion is a major problem in patients undergoing HSCT. In a cohort of 50 pediatric patients affected by beta thalassemia coming from Middle East countries, we experienced a high incidence of refractoriness because of anti-HLA antibodies during post-HSCT aplasia. In a risk factors analysis, factors predicting a negative transfusion outcome were presence of spleen and the number of anti-HLA antibodies. We adopted a policy to select platelet donors by avoiding HLA antigens against which the patient had specific antibodies. Transfusion of dedicated units resulted in 26% refractoriness compared to 74% to random units (p < 0.0001). When dedicated transfusions were used, the presence of spleen did not influence transfusion outcome. Analyzing transfusion outcome depending on the degree of HLA match and ABO compatibility, 76% successful transfusions were obtained with HLA-matched- ABO compatible followed by 67% in HLA-1mismatch- ABO compatible or HLA-matched- ABO incompatible and by 46% in HLA-1mismatch- ABO incompatible. In conclusion, we provide evidence that the selection of platelet donors according to patient characteristics, anti-HLA antibodies and ABO matching, is successful in reducing platelet refractoriness in heavily alloimmunized thalassemia patients undergoing transplantation.
机译:免疫介导的血小板输注难治性是接受HSCT的患者的主要问题。在来自中东国家的50名受β地中海贫血影响的儿科患者队列中,由于在HSCT发育不全期间出现抗HLA抗体,导致难治性发生率很高。在危险因素分析中,预测阴性输血结果的因素是脾脏的存在和抗HLA抗体的数量。我们采取了一项避免使用HLA抗原来选择血小板供体的政策,因为该抗原具有患者的特异性抗体。输注专用单位的耐火度为26%,而随机单位为74%(p <0.0001)。使用专用输血时,脾脏的存在不会影响输血结果。根据HLA匹配程度和ABO相容性来分析输血结果,与HLA匹配的ABO相容性成功输血的比例为76%,其次为HLA-1不匹配的ABO相容性或HLA匹配的ABO不相容性的成功输血率为67%。 HLA-1不匹配-ABO不兼容。总之,我们提供的证据表明,根据患者特征,抗HLA抗体和ABO匹配选择血小板供体可以成功降低接受移植的重度同种免疫性地中海贫血患者的血小板不应性。

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