主要ABO血型不合异基因造血干细胞移植后纯红细胞再生障碍

摘要

Six out of 20 patients undergoing a major ABO-incompatible allogeneic stem cell transplantation(alloHSCT) developed pure red cell aplasia (PRCA), which did not show any effects on granulocyte and platelet engraftment, and incidence of grade Ⅱ - Ⅳ aGVHD, All the 6 cases of PRCA were in blood group O recipients of grafts from blood group A donors( n = 5) or blood group B donor( n = 1 ), suggesting that donor/recipient pair(A/O) is associated with a high risk of PRCA after major ABO-incompatible allo-HSCT. Erythroid engraftment occurred spontaneously in four cases without specific intervention other than the RBC transfusion, which coincided with the decrease of isoagglutinin titers below 8, and the remaining 2 patients with prolonged erythroid aplasia( ＞300 days)despite therapy with erythropoietin (EPO) were successfully treated by plasma exchange with donor-type plasma replacement. Cyclosporine did not appear to have played any role in causing PRCA in our patients, however, the occurrence of GVHD may facilitate the recovery of erythropoiesis.%20例主要ABO血型不合异基因造血干细胞移植(allo-HSCT)患者中,6例发生纯红细胞再生障碍(PRCA).PRCA对中性粒细胞和血小板植入以及Ⅱ-Ⅳ度aGVHD并无影响.6例PRCA患者血型均为O型,而供者血型5例为A型,1例B型,提示供/受者血型A/O是主要ABO血型不合allo-HSCT后PRCA发生的高危因素.4例除给予RBC输注无其它特殊治疗,随着凝集素滴度降至＜8,红系造血自然恢复,而另2例尽管给予重组人红细胞生成素(rhEPO)治疗红系再障仍持续＞300天,经供者型血浆置换而红系恢复造血.在本组病例,环孢菌素在PRCA发生中并无作用,而GVHD发生则可促进红系造血恢复.