造血干细胞移植治疗重症再生障碍性贫血

Haemopoietic Stem Cell Transplantation for Treatment of Severe Aplastic Anemia

分别采用同基因ＢＭＴ及异基因ＢＭＴ治愈重症再生障碍性贫血（ＳＡＡ）各１例。例１经各种基因标志分析识别其与孪生兄弟为同卵双生，故采用单纯输注骨髓辅以ＧＭＣＳＦ，使其治愈，已逾４年。例２经ＨＬＡ血清学、细胞学和ＤＲＢＰＣＲ／ｐｒｉｎｔｉｎｇ配型证实与其弟ＨＬＡ基因型相合，以Ｃｙ＋ＡＴＧ为预处理方案，移植骨髓细胞及外周血干细胞，用ＣＳＰ＋ＭＴＸ预防ＧｖＨＤ，移植物于＋１４ｄ植活，＋６１ｄ其ＲＢＣ抗原由ＭＮ转为ＮＮ供者型，现正在康复之中。

Two patients with severe aplastic anemia(SAA) treated by syngeneic BMT and allogeneic BMT plus peripheral blood stem cell transplantation (PBSCT) respectively were reported. The patient suffering from SAA Ⅱ had an identical twin brother. He received brother's marrow transfusion (1.5×10 8/kg) alone and had sustained recovery of PB counts and marrow haemopoiesis.He is alive and works normally more than 4 years. Another patient with very SAA(vSAA) and had an HLA full matched(HLA typing, cross matched MLC and DRB PCR/printing) brother as donor. He had received previous transfusion 12 times. He was administered four daily doses of Cy (50 mg/kg×4, total 200 mg/kg) alternating with three doses of ATG (25 mg/kg×3, total 75 mg/kg) followed by a HLA identical marrow graft ( 2.7×10 8 /kg,0 d) and peripheral blood stem cell ( PBSC ) transfusion ( CD34 +cells 3.85×10 5/kg,+1 d). Methotrexate (MTX) and cyclosporine A(CSP) were administered to prevent GvGH. His PMNs increased more than 0.2×10 9/L since +14 days and RBC antigen from MN became NN (donor′s source ) at +61 d. However,he was complicated with systemic MRSA infection since +12 d and followed by abscess of thoracic vertebra.The managements for abcess were effective and the patient is recovering. The article also discussed some problems with treatment of SAA.