Bélanger R, Gyger M, Perreault C, Bonny Y, St-Louis J
Department of Haematology, Maisonneuve-Rosemont Hospital, Montreal, Quebec, Canada.
Br J Haematol. 1988 May;69(1):29-33. doi: 10.1111/j.1365-2141.1988.tb07598.x.
Eight patients with myelodysplastic syndromes (MDS) were treated with bone marrow transplantation (BMT). Median age was 34.5 years and ranged between 3 and 45. FAB diagnosis was refractory anaemia (RA) in three, RA with excess of blasts (RAEB) in four and RAEB in transformation (RAEB-t) in one case. Four patients were prepared with cyclophosphamide and total body irradiation whilst the other four received busulphan and cyclophosphamide. Engraftment was documented in seven of eight patients. Two patients died from complications related to the procedure. One had early veno-occlusive disease of the liver whilst the other died 46 months after BMT from pulmonary fibrosis. One patient died from recurrent disease 11 months after BMT. Five patients are alive and in complete remission 9-35 months post-transplantation. Four of these patients have a Karnofsky score greater than or equal to 90%. These results suggest that BMT can induce prolonged disease-free survival in patients under 50 years of age. If a compatible donor is available, marrow transplantation should be seriously considered in the treatment of MDS.
8例骨髓增生异常综合征（MDS）患者接受了骨髓移植（BMT）治疗。中位年龄为34.5岁，范围在3岁至45岁之间。FAB诊断为3例难治性贫血（RA）、4例伴有过多原始细胞的难治性贫血（RAEB）和1例转化中的RAEB（RAEB-t）。4例患者采用环磷酰胺和全身照射进行预处理，另外4例接受白消安和环磷酰胺。8例患者中有7例记录到植入成功。2例患者死于与该手术相关的并发症。1例患有早期肝静脉闭塞病，另1例在BMT后46个月死于肺纤维化。1例患者在BMT后11个月死于疾病复发。5例患者存活，移植后9至35个月处于完全缓解状态。其中4例患者的卡氏评分大于或等于90%。这些结果表明，BMT可使50岁以下患者获得长期无病生存。如果有合适的供体，在MDS的治疗中应认真考虑骨髓移植。
