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(英文)

Pure Red Cell Aplasia Following Major ABO-Incompatible Allogeneic Hematopoietic Stem Cell Transplantation

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 >30 0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 。

Six out of 20 patients undergoing a major ABO incompatible allogeneic stem cell transplantation(allo HSCT) 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.