Marsh J C, Harhalakis N, Dowding C, Laffan M, Gordon-Smith E C, Hows J M
Paterson Institute for Cancer Research, Christie Hospital, Manchester, UK.
Bone Marrow Transplant. 1989 Sep;4(5):581-5.
We present the case of a 60-year-old woman with drug-induced aplastic anaemia with a healthy monozygotic twin. Proof of monozygosity was confirmed by studies using the hypervariable minisatellite probe to obtain identical DNA fingerprints in donor and recipient. In vitro co-culture studies performed showed no evidence of a recipient-derived cellular or humoral inhibitor of donor haemopoiesis. Despite this, there was no engraftment following simple marrow infusion without preconditioning. A second syngeneic transplant following high dose cyclophosphamide therapy produced trilineage engraftment but severe thrombocytopenia developed at 3 months, followed later by pancytopenia with generalized marrow failure. Following a third syngeneic transplant with cyclophosphamide and total lymphoid irradiation there was good initial engraftment but graft failure occurred at 14 weeks. A fourth transplant using Campath 1G as preconditioning resulted in no engraftment and the patient died of septicaemia 8 weeks following her fourth transplant. We suggest that the cause of the recurrent aplastic anaemia in this case was a defect of marrow stroma as neither an inhibitor of donor haemopoiesis nor an intrinsic defect of donor stem cell growth could be demonstrated in vitro.
我们报告了一例60岁患有药物性再生障碍性贫血的女性病例,她有一个健康的同卵双胞胎。通过使用高变微卫星探针进行研究,证实了供体和受体的DNA指纹相同,从而确认了同卵性。进行的体外共培养研究没有发现受体来源的供体造血细胞或体液抑制剂的证据。尽管如此,在没有预处理的情况下进行简单的骨髓输注后并没有发生植入。在高剂量环磷酰胺治疗后进行的第二次同基因移植产生了三系植入,但在3个月时出现了严重的血小板减少,随后出现全血细胞减少和全身性骨髓衰竭。在进行环磷酰胺和全身淋巴照射的第三次同基因移植后,最初植入良好,但在14周时出现移植失败。使用Campath 1G作为预处理的第四次移植没有导致植入,患者在第四次移植后8周死于败血症。我们认为,该病例中复发性再生障碍性贫血的原因是骨髓基质缺陷,因为在体外既未证明存在供体造血抑制剂,也未证明存在供体干细胞生长的内在缺陷。