Division of Hematology, The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.
Blood. 2013 Aug 8;122(6):1062-71. doi: 10.1182/blood-2013-03-490623. Epub 2013 May 30.
Red blood cell (RBC) transfusion is a key treatment of patients with sickle cell disease (SCD) but remains complicated by RBC immunization. In the present study, we evaluated the effects of antigen matching for Rh D, C, and E, and K and transfusion from African American donors in 182 patients with SCD. Overall, 71 (58%) chronic and 9 (15%) episodically transfused patients were alloimmunized. Fifty-five (45%) chronic and 7 (12%) episodically transfused patients were Rh immunized. Of 146 antibodies identified, 91 were unexplained Rh antibodies, one-third of which were associated with laboratory evidence of delayed transfusion reactions. Fifty-six antibodies occurred in patients whose RBCs were phenotypically positive for the corresponding Rh antigen and 35 in patients whose RBCs lacked the antigen and were transfused with Rh-matched RBCs. High-resolution RH genotyping revealed variant alleles in 87% of individuals. These data describe the prevalence of Rh alloimmunization in patients with SCD transfused with phenotypic Rh-matched African American RBCs. Our results suggest that altered RH alleles in both the patients and in the donors contributed to Rh alloimmunization in this study. Whether RH genotyping of patients and minority donors will reduce Rh alloimmunization in SCD needs to be examined.
红细胞（RBC）输血是治疗镰状细胞病（SCD）患者的关键方法，但仍然存在 RBC 免疫的问题。在本研究中，我们评估了 Rh D、C、E 和 K 抗原匹配以及来自非裔美国供体的输血对 182 例 SCD 患者的影响。总体而言，71 例（58%）慢性输血患者和 9 例（15%）间歇性输血患者发生同种免疫。55 例（45%）慢性输血患者和 7 例（12%）间歇性输血患者发生 Rh 免疫。在鉴定的 146 种抗体中，有 91 种是无法解释的 Rh 抗体，其中三分之一与实验室证据表明存在迟发性输血反应有关。56 种抗体出现在 RBC 表型阳性的患者中，这些患者的 RBC 缺乏相应的 Rh 抗原，且接受了 Rh 匹配的 RBC 输血。高分辨率 RH 基因分型显示，87%的个体存在变异等位基因。这些数据描述了用表型 Rh 匹配的非裔美国 RBC 输血的 SCD 患者中 Rh 同种免疫的流行情况。我们的结果表明，患者和供体中的改变的 RH 等位基因导致了本研究中的 Rh 同种免疫。对患者和少数族裔供体进行 RH 基因分型是否会减少 SCD 中的 Rh 同种免疫，仍需进一步研究。
