Laboratory of Transfusion Biology, Department of Pathology and Cell Biology, Columbia University Irving Medical Center and New York Presbyterian Hospital, New York, New York.
Aflac Cancer and Blood Disorders Center, Children's Healthcare of Atlanta.
Curr Opin Hematol. 2019 Nov;26(6):434-441. doi: 10.1097/MOH.0000000000000540.
Red blood cell (RBC) transfusion is an important treatment for some complications of sickle cell disease (SCD). On the contrary, transfusion may lead to alloimmunization to RBC antigens, with such alloantibodies putting patients at risk for acute or delayed hemolysis, and increasing the difficulty of finding compatible RBCs. Patients with SCD are more susceptible to developing RBC alloantibodies than other multiply transfused patient populations, for reasons that are not completely understood. In this review, we summarize the available data about risk factors and underlying mechanisms associated with RBC alloimmunization in SCD.
Although RBC antigen matching between blood donors and transfusion recipients can decrease alloimmunization, complete matching at all loci is not feasible. Patients with SCD show evidence of increased inflammation at baseline and in times of illness. Resultant changes to the innate and adaptive immune systems presumably influence the development of RBC alloantibodies as well as RBC autoantibodies.
The inflammation and immune dysregulation associated with SCD may be therapeutic targets for preventing the formation of antibodies and/or for mitigating the dangers of existing RBC alloantibodies. As long as RBC transfusion therapy remains an important treatment for SCD, the quest to improve its safety profile will continue.
红细胞（RBC）输血是治疗镰状细胞病（SCD）某些并发症的重要手段。然而，输血可能导致 RBC 抗原的同种免疫，导致同种抗体使患者面临急性或迟发性溶血的风险，并增加寻找相容 RBC 的难度。由于尚未完全了解，SCD 患者比其他多次输血患者更容易产生 RBC 同种抗体。在这篇综述中，我们总结了有关 SCD 中与 RBC 同种免疫相关的危险因素和潜在机制的现有数据。
尽管献血者和输血受者之间的 RBC 抗原匹配可以降低同种免疫，但在所有位点完全匹配是不可行的。SCD 患者在基线和患病时均表现出炎症增加的证据。先天和适应性免疫系统的变化可能会影响 RBC 同种抗体以及 RBC 自身抗体的产生。
与 SCD 相关的炎症和免疫失调可能是预防抗体形成和/或减轻现有 RBC 同种抗体危险的治疗靶点。只要 RBC 输血治疗仍然是 SCD 的重要治疗手段，提高其安全性的探索就会继续。
