Yazdanbakhsh Karina
Complement Biology, New York Blood Center, New York, NY 10021, USA.
Curr Opin Hematol. 2005 Mar;12(2):117-22. doi: 10.1097/01.moh.0000151712.53957.01.
Complement sensitization of red blood cells (RBCs) can lead to both intravascular and extravascular red cell destruction. Altered levels of naturally occurring complement regulatory proteins on red cells can result in hemolysis, while defective expression of these proteins on immune cells can cause breakdown of tolerance to self antigens and is associated with autoimmune disease.
To date several complement inhibitors, including recombinant forms of complement regulatory proteins, humanized antibodies, and synthetic molecules have been described that limit complement activation by interfering with different steps in the complement cascade. However, few have been evaluated for prevention of complement-mediated RBC destruction. In this review, possible applications of these complement inhibitors for treatment of complement-mediated hemolysis in specific disease states are described. Furthermore, the implication of the regulatory role of complement in the development of autoimmune hemolytic anemia is discussed.
Complement therapeutics has potential for effective and safe prophylactic use and treatment of hemolytic transfusion reactions and complement-mediated hemolytic diseases. Furthermore, the regulatory function of complement may be exploited to prevent and treat autoimmune hemolytic anemia.
红细胞（RBC）的补体致敏可导致血管内和血管外红细胞破坏。红细胞上天然存在的补体调节蛋白水平改变可导致溶血，而这些蛋白在免疫细胞上的表达缺陷可导致对自身抗原的耐受性破坏，并与自身免疫性疾病相关。
迄今为止，已经描述了几种补体抑制剂，包括补体调节蛋白的重组形式、人源化抗体和合成分子，它们通过干扰补体级联反应的不同步骤来限制补体激活。然而，很少有药物被评估用于预防补体介导的红细胞破坏。在这篇综述中，描述了这些补体抑制剂在特定疾病状态下治疗补体介导的溶血的可能应用。此外，还讨论了补体的调节作用在自身免疫性溶血性贫血发生发展中的意义。
补体疗法在有效、安全地预防和治疗溶血性输血反应及补体介导性溶血性疾病方面具有潜力。此外，补体的调节功能可用于预防和治疗自身免疫性溶血性贫血。
