亚历山大大学儿童医院输血依赖性β-地中海贫血患者的红细胞异体免疫

Nehad Hassanein, Bothaina El-Domiaty, Rovaida El-Fawal, Nevien Mikhail
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Transfusion and clinical records of the latter were reviewed to assess the diagnosis of β-thalassemia, the age of the first presentation, the age at which transfusion therapy was started, transfusion interval, and the number of red cell units given. Alloantibody detection was then performed. Results In the present study, 11 (11%) out of the total 100 studied patients were alloimmunized. The total number of alloantibodies identified in these patients was 25. Two patients (18%) had only one alloantibody, six patients (54.5%) had two alloantibodies, one patient (9%) had three alloantibodies, and two patients (18%) had more than three alloantibodies. The most prevalent antibodies belonged to Kell and Rh blood-group systems (seven out of 25 each). Conclusions When it comes to thalassemia patients dependent on blood transfusion, red blood cell alloimmunization is a difficult complication. Kell and RH blood-group-system antibodies are the predominant antibodies. 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摘要

在埃及,已经确定最常见的遗传性血红蛋白疾病是β-地中海贫血。这种疾病的严重形式的管理主要包括红细胞输注。有关病人定期接受这些输血。因此,红细胞异体抗体和自身抗体的形成是不可避免的。这项研究解决了这一难题,旨在确定输血依赖性ß-地中海贫血儿童中红细胞同种免疫的流行程度,这些儿童经常在亚历山大大学儿童医院血液学诊所接受治疗。患者与方法本研究共筛选100例输血依赖患者,年龄均为3 ~ 16岁。输血和后者的临床记录进行审查,以评估β-地中海贫血的诊断,第一次表现的年龄,输血治疗开始的年龄,输血间隔时间和给予的红细胞单位数。然后进行同种异体抗体检测。结果100例患者中有11例(11%)实现同种异体免疫。在这些患者中发现的同种异体抗体总数为25个。2例(18%)患者仅有1种同种异体抗体,6例(54.5%)患者有2种同种异体抗体,1例(9%)患者有3种同种异体抗体,2例(18%)患者有3种以上同种异体抗体。最普遍的抗体属于凯尔和Rh血型系统(25个中各有7个)。结论对于依赖输血的地中海贫血患者,红细胞同种异体免疫是一个困难的并发症。Kell和RH血型系统抗体是主要抗体。同种异体抗体的形成主要取决于接受的红细胞单位的性质以及供体和受体因素。这可以解释不同报告中同种免疫接种率的差异。从长远来看,避免同种异体免疫可以降低治疗成本,提高患者的生活质量。
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Red cell alloimmunization in transfusion-dependent β-thalassemia patients attending Alexandria University Children Hospital
Background In Egypt, it has been established that the most frequently inherited disorder of hemoglobin is β-thalassemia. Management of the severe forms of this disorder consists chiefly of red cell transfusion. The concerned patients receive these transfusions on a regular basis. Therefore, the formation of antired cell alloantibodies and autoantibodies was inevitable. This dilemma is addressed in this study that aims to determine the prevalence of red cell alloimmunization among transfusion-dependent ß-thalassemia children that receive frequent care in the Hematology Clinic of Alexandria University Children’s Hospital. Patients and methods This study screened 100 transfusion-dependent patients in total, all of them were 3–16 years old. Transfusion and clinical records of the latter were reviewed to assess the diagnosis of β-thalassemia, the age of the first presentation, the age at which transfusion therapy was started, transfusion interval, and the number of red cell units given. Alloantibody detection was then performed. Results In the present study, 11 (11%) out of the total 100 studied patients were alloimmunized. The total number of alloantibodies identified in these patients was 25. Two patients (18%) had only one alloantibody, six patients (54.5%) had two alloantibodies, one patient (9%) had three alloantibodies, and two patients (18%) had more than three alloantibodies. The most prevalent antibodies belonged to Kell and Rh blood-group systems (seven out of 25 each). Conclusions When it comes to thalassemia patients dependent on blood transfusion, red blood cell alloimmunization is a difficult complication. Kell and RH blood-group-system antibodies are the predominant antibodies. Formation of alloantibodies depends mainly on the nature of red cell units received and donor and recipient factors. These can explain the difference in alloimmunization rates in different reports. Avoidance of alloimmunization would decrease the cost of treatment on the long run and improve the patient quality of life.
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