同种异体骨髓移植治疗10例重型再生障碍性贫血患者的远期随访资料分析

目的：提高重型再生障碍性贫血(SAA)患者的长期治愈率。方法：1980年5月一2001年12月采用同种异体骨髓移植(BMT)治疗SAA患者l0例，并对其临床及远期随访资料进行分析。结果：近期死亡4例，转入侵性再生障碍性贫血(CAA)3例，治愈3例。治愈的3例中，l例8年后出现骨髓增生异常综合征(MDS)，以后转变成急性单核细胞白血病。结论：同种异体BMT是治疗SAA的有效治疗方法。加强对感染的防治和移植物抗宿主病(GVHD)的控制是移植后早期阶段的治疗关键。SAA经BMT治疗后的随访结果提示，BMT中的预处理方案有必要加以改进，应作染色体及基因检测，以探讨基因突变是否是AA转化为AC的机制。

Clinical analysis and longterm follow-up of 10 cases of severe aplastic anemia receiving Auo-BMT

Objective:In order to improve the cure rate of SAA.Method:Clinical analysis and long term follow up on 10 cases of SAA receiving allo BMT from May 1980 to Dec 2001.Result:4 patients died at early stage, 3 patients turned to CAA and 3 Patients were cured. One of the 3 patients who were cured developed MDS after 8 years,and then turned to AML later.Conclusion:BMT is still an effective method for SAA treatment. The key of the early stage after BMT is to prevent and treat infection,and to control GVHD. Although some of the SAA patients failed in the transplantation,they turned to CAA and got a long time survival. One of the 3 patients who were cured developed into MDS after 8 years,then turned to AML, the mechanism may relates to the immune system defect caused by cytotoxic drugs in transplant pretreatment, or to the abnormal stem cells in SAA. So it puts forword a new problem and challenge for the future BMT in SAA.