Department of Clinical Hematology, Hospital Clínic, IDIBAPS, Barcelona, Spain.
Transfus Med Rev. 2013 Jul;27(3):166-70. doi: 10.1016/j.tmrv.2013.02.004. Epub 2013 Apr 4.
Immune hemolytic anemia is a well-recognized complication after allogeneic hematopoietic stem cell transplantation (HSCT). There are 4 possible causes for this complication. First, antibodies present in the recipient destroy donor cells. Second, donor red cell antibodies at the time of stem cell infusion are transferred to the recipient. Third, sometimes, engrafted donor lymphocytes cause active production of red cell antibodies. Fourth, another cause of hemolysis after allogeneic HSCT is autoimmune hemolytic anemia (AIHA). It is thought to be due to antibodies produced by the donor's immune system against antigens on red cells of donor origin. Autoimmune hemolytic anemia after allogeneic HSCT is rare, it is still not well characterized, and it represents a life-threatening situation. We describe 2 patients with acute myeloid leukemia treated with intensive chemotherapy and umbilical cord blood stem cell transplantation (UCBT). One patient developed AIHA at day +182 and the other at day +212 after receiving UCBT. Patients received 5 and 7 line treatment options, respectively, including continuous corticosteroids, intravenous immunoglobulin, splenectomy, cyclophosphamide, plasma exchange, rituximab, bortezomib, and eculizumab. However, both patients died because of massive hemolysis after 85 and 106 days of intensive treatment, respectively. These cases reflect the extreme difficulty in the therapeutic management of patients with AIHA following UCBT. After an extensive review of the literature, the exact physiopathologic mechanisms of AIHA after allogeneic HSCT in general, and after UCBT in particular, and therefore an effective treatment remain unknown.
免疫性溶血性贫血是异基因造血干细胞移植（HSCT）后一种公认的并发症。这种并发症有 4 个可能的原因。首先，受体内存在的抗体破坏供体细胞。其次，干细胞输注时供体红细胞抗体转移到受体内。第三，有时，植入的供体细胞淋巴细胞会导致红细胞抗体的主动产生。第四，异基因 HSCT 后溶血的另一个原因是自身免疫性溶血性贫血（AIHA）。它被认为是由于供体免疫系统产生的抗体针对供体来源的红细胞抗原。异基因 HSCT 后的自身免疫性溶血性贫血很少见，尚未得到很好的描述，并且代表着危及生命的情况。我们描述了 2 例接受强化化疗和脐带血干细胞移植（UCBT）治疗的急性髓系白血病患者。一名患者在接受 UCBT 后第 182 天发生 AIHA，另一名患者在第 212 天发生 AIHA。患者分别接受了 5 线和 7 线治疗选择，包括持续皮质类固醇、静脉注射免疫球蛋白、脾切除术、环磷酰胺、血浆置换、利妥昔单抗、硼替佐米和依库珠单抗。然而，由于强化治疗后 85 天和 106 天分别发生大量溶血，两名患者均死亡。这些病例反映了 UCBT 后 AIHA 患者治疗管理的极端困难。在广泛回顾文献后，一般来说，异基因 HSCT 后特别是 UCBT 后 AIHA 的确切病理生理机制以及有效的治疗方法仍不清楚。
