Furuya H, Wakayama T, Tanaka J, Tsumori M, Hatazoe T, Ohguni S, Sato T, Ishikura H, Kato Y
First Division, Department of Medicine, Shimane Medical University, Japan.
Rinsho Ketsueki. 1995 Nov;36(11):1279-83.
Pure red cell aplasia (PRCA) was found in a male patient with chronic myelocytic leukemia after major ABO incompatible bone marrow transplantation (BMT). He had blood group O, and received BMT from an HLA identical sibling (blood group A). Erythrocyte-depleted marrow was transplanted. Methotrexate for short time and cyclosporine (CyA) were used for graft versus host disease (GVHD) prophylaxis. Engraftment of neutrophils and platelets were observed on day 14 and 22, respectively. The Ph1 chromosome disappeared on day 133. However engraftment of erythrocytes was not observed on day + 280. Bone marrow puncture revealed depletion of erythrocyte precursors. Anti-A isoagglutinin was persisted. There was no evidence of acute or chronic GVHD. Administration of prednisolone, discontinuance of CyA and subcutaneous infusion of recombinant human erythropoietin failed to improve PRCA. Bolus methylprednisolone (m-PSL) therapy started on day 284 resulted in rapid increase in reticulocyte counts within 6 days, which was followed by normal hemoglobin concentrations. We conclude that bolus m-PSL may be one treatment for PRCA after BMT.
一名慢性粒细胞白血病男性患者在进行主要ABO血型不相合的骨髓移植（BMT）后发生了纯红细胞再生障碍性贫血（PRCA）。他血型为O型，接受了来自一名HLA相合同胞（血型为A型）的骨髓移植。移植的是去除红细胞的骨髓。短期使用甲氨蝶呤和环孢素（CyA）预防移植物抗宿主病（GVHD）。分别在第14天和第22天观察到中性粒细胞和血小板植入。第133天Ph1染色体消失。然而，在+280天时未观察到红细胞植入。骨髓穿刺显示红细胞前体细胞减少。抗A同种凝集素持续存在。没有急性或慢性GVHD的证据。给予泼尼松龙、停用CyA以及皮下注射重组人促红细胞生成素均未能改善PRCA。第284天开始的大剂量甲泼尼龙（m-PSL）治疗导致网织红细胞计数在6天内迅速增加，随后血红蛋白浓度恢复正常。我们得出结论，大剂量m-PSL可能是BMT后PRCA的一种治疗方法。
