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基于 DNA 分型的临床影响和为慢性输血地中海贫血患者提供抗原匹配的红细胞单位。

Clinical impacts of DNA-based typing and provision of antigen-matched red blood cell units for chronically transfused patients with thalassemia.

机构信息

Medicine, Faculty of Medicine, Chulalongkorn University, Transfusion Medicine Unit, King Chulalongkorn Memorial Hospital, Research Unit in Translational Hematology, Division of Hematology, Department of Medicine, Faculty of Medicine, Chulalongkorn University.

Specialist in Transfusion Medicine, Transfusion Medicine Unit, King Chulalongkorn Memorial Hospital, Bangkok, Thailand.

出版信息

Immunohematology. 2020 Dec;36(4):137-145.

Abstract

Blood transfusion, the main therapy for patients with severe thalassemia, is challenged by alloantibodies that can lead to hemolytic transfusion reactions. The use of prophylactic antigen-matched units is recommended, but serologic typing, before the first transfusion, is rarely performed and is not reliable after chronic transfusion. Patient DNA-based typing is a promising strategy, but clinical outcome data are lacking. The aim of this study was to determine the benefits of antigenmatched transfusion guided by DNA-based typing in terms of new alloantibody formation and increases in mean pretransfusion hemoglobin (Hb) levels. We performed DNA-based typing on samples from 24 transfusion-dependent patients with thalassemia who had no serologic phenotyping performed before the first transfusion. These patients were then transfused with antigen-matched donor RBC units that were typed serologically. New alloantibody formation and mean pre-transfusion Hb levels were evaluated after implementing this extended common antigen-matching transfusion protocol. Sixty-three percent of the patients in this study were diagnosed as having beta-thalassemia Hb E. Alloantibodies were already present in 87.5 percent (21/24) of these patients, and most of these antibodies were multiple and/ or unidentified. After the enrollment, there were 717 transfusion episodes comprising 1209 RBC units. The number of RBC units transfused to each patient ranged from 22 to 119 units. At the median duration of 25.5 months (range 10-34 months), no new alloantibodies were detected since the beginning of the protocol. Seventy-four transfusion episodes in six patients were crossmatch-positive due to autoantibodies (patients 2, 4, 8, 9, and 14) or anti-Chido (patient 18) that had been identified before the study. There were no hemolytic transfusion reactions in this study. Five patients (patients 1, 2, 12, 15, and 20) showed increased mean pre-transfusion Hb levels (≥1 g/dL) and one patient (patient 16) had longer intervals between transfusions (compared with those before the protocol), suggesting longer RBC survival, although there was no statistical difference in the whole group. Our study highlights the benefits of DNA-based typing in chronically transfused patients with thalassemia who had no phenotyping data before the first transfusion. Patient DNA-based typing for antigen-matched transfusion is safe in thalassemia and allows us to obtain better-matched blood units for complicated patients.

摘要

输血是治疗重症地中海贫血患者的主要方法,但会受到同种异体抗体的挑战,从而导致溶血性输血反应。建议使用预防性抗原匹配的单位,但在第一次输血前很少进行血清学分型,而且在慢性输血后也不可靠。基于患者 DNA 的分型是一种很有前途的策略,但缺乏临床结果数据。本研究旨在确定基于 DNA 的分型指导下的抗原匹配输血在新的同种异体抗体形成和平均输血前血红蛋白 (Hb) 水平升高方面的益处。我们对 24 名依赖输血的地中海贫血患者的样本进行了 DNA 分型,这些患者在第一次输血前没有进行血清表型分析。然后,我们用经过血清学检测的抗原匹配供者 RBC 单位对这些患者进行输血。在实施这种扩展的常见抗原匹配输血方案后,评估新的同种异体抗体形成和平均输血前 Hb 水平。本研究中的 63%的患者被诊断为β-地中海贫血 Hb E。这些患者中有 87.5%(21/24)已经存在同种异体抗体,而且这些抗体大多是多种和/或未识别的。入组后,共进行了 717 次输血,包括 1209 个 RBC 单位。每位患者接受的 RBC 单位数量从 22 到 119 个不等。在中位 25.5 个月(范围 10-34 个月)的随访期间,自方案开始以来未检测到新的同种异体抗体。由于自身抗体(患者 2、4、8、9 和 14)或抗 Chido(患者 18),在研究前已确定的 6 名患者的 74 次输血交叉配血阳性。本研究中没有发生溶血性输血反应。5 名患者(患者 1、2、12、15 和 20)的平均输血前 Hb 水平升高(≥1 g/dL),1 名患者(患者 16)的输血间隔延长(与方案前相比),提示 RBC 存活时间延长,尽管整个组没有统计学差异。本研究强调了在第一次输血前没有表型数据的慢性输血地中海贫血患者中进行基于 DNA 的分型的益处。对于地中海贫血患者,进行基于患者 DNA 的抗原匹配输血是安全的,并且可以为复杂患者获得更好匹配的血液单位。

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