RH genotypes and red cell alloimmunization rates in chronically transfused patients with sickle cell disease: A multisite study in the USA.
RH genotypes and red cell alloimmunization rates in chronically transfused patients with sickle cell disease: A multisite study in the USA.
复制标题
长期输血镰状细胞病患者的 RH 基因型和红细胞同种免疫率：美国的一项多中心研究。
DOI：
10.1111/trf.17740
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发表时间：
2024
期刊：
影响因子：
2.9
通讯作者：
Chou,StellaT
中科院分区：
文献类型：
--
作者：
Israelyan,Narek;Vege,Sunitha;Friedman,DavidF;Zhang,Zhe;Uter,Stacey;Fasano,RossM;Yee,Marianne;Piccone,Connie;Kelly,Shannon;Hankins,JaneS;Zheng,Yan;Westhoff,ConnieM;Chou,StellaT
红细胞同种异体免疫对于镰状细胞病（SCD）患者仍然是一个挑战，并导致溶血性输血反应和相关合并症的风险增加。尽管ABO，Rh和K的预防性血清学匹配，红细胞同种异体免疫持续存在，部分原因是由于高频率的variantRH等位基因在SCD患者和黑人Black blood donators.Study Design and MethodsWe比较RH基因型和allimmunization率在342名儿童和年轻成人SCD患者慢性输血治疗暴露于> 90，000红细胞单位在5个地点在美国。RHD和RHCEBeadChip阵列和靶向assays.ResultsPrevalence进行基因分型整体和Rh特异性同种异体免疫的机构之间的变化，范围从5%到41%（p= 0.0035）和5%-33%（p= 0.0002），respectively。RH基因分型表明，33%RHD和57%RHCE等位基因在这个队列变异。携带编码部分e抗原的RHCE等位基因的患者比编码至少一种常规e抗原的患者有更高的抗e检出率（p= .0007）。与传统抗原相比，预测部分或改变抗原表达的患者中抗D、抗C或抗E形成无差异，这表明供体细胞上的变异Rh也可能刺激对这些抗原的同种免疫。讨论这些结果突出了同种免疫率的变化，并表明Rh抗原匹配的分子方法可能是必要的，以最佳预防同种免疫，在患者和黑人供体中变异RH等位基因的高患病率。
BackgroundRed cell alloimmunization remains a challenge for individuals with sickle cell disease (SCD) and contributes to increased risk of hemolytic transfusion reactions and associated comorbidities. Despite prophylactic serological matching for ABO, Rh, and K, red cell alloimmunization persists, in part, due to a high frequency of variantRHalleles in patients with SCD and Black blood donors.Study Design and MethodsWe comparedRHgenotypes and rates of alloimmunization in 342 pediatric and young adult patients with SCD on chronic transfusion therapy exposed to >90,000 red cell units at five sites across the USA. Genotyping was performed withRHDandRHCEBeadChip arrays and targeted assays.ResultsPrevalence of overall and Rh‐specific alloimmunization varied among institutions, ranging from 5% to 41% (p= .0035) and 5%–33% (p= .0002), respectively.RHgenotyping demonstrated that 33%RHDand 57%RHCEalleles were variant in this cohort. Patients withRHCEalleles encoding partial e antigens had higher rates of anti‐e identified than those encoding at least one conventional e antigen (p= .0007). There was no difference in anti‐D, anti‐C, or anti‐E formation among patients with predicted partial or altered antigen expression compared to those with conventional antigens, suggesting that variant Rh on donor cells may also stimulate alloimmunization to these antigens.DiscussionThese results highlight variability in alloimmunization rates and suggest that a molecular approach to Rh antigen matching may be necessary for optimal prevention of alloimmunization given the high prevalence of variantRHalleles among both patients and Black donors.