Yusuf U, Frangoul H A, Gooley T A, Woolfrey A E, Carpenter P A, Andrews R G, Deeg H J, Appelbaum F R, Anasetti C, Storb R, Sanders J E
Fred Hutchinson Cancer Research Center and University of Washington Department of Pediatrics, Seattle, WA 98109, USA.
Bone Marrow Transplant. 2004 Apr;33(8):805-14. doi: 10.1038/sj.bmt.1704438.
The purpose of this study was to evaluate the role of allogeneic bone marrow transplantation (BMT) in children with myelodysplastic syndrome (MDS). In total, 94 consecutive pediatric patients with MDS received an allogeneic BMT from 1976 to 2001 for refractory anemia (RA) (n=25), RA with ringed sideroblasts (RARS) (n=2), RA with excess blasts (RAEB) (n=20), RAEB in transformation (RAEB-T) (n=14), juvenile myelomonocytic leukemia (JMML) (n=32) or chronic myelomonocytic leukemia (CMML) (n=1). The estimated 3-year probabilities of survival, event-free survival (EFS), nonrelapse mortality and relapse were 50, 41, 28 and 29%, respectively. Patients with RA/RARS had an estimated 3-year survival of 74% compared to 68% in those with RAEB and 33% in patients with JMML/CMML. In multivariable analysis, patients with RAEB-T or JMML were 3.9 and 3.7 times more likely to die compared to those with RA/RARS and RAEB (P=0.005 and 0.004, respectively). Patients with RAEB-T were 5.5 times more likely to relapse (P=0.01). The median follow-up among the 43 surviving patients is 10 years (range 1-25). We conclude that allogeneic BMT for children with MDS is well tolerated and can be curative.
本研究的目的是评估异基因骨髓移植(BMT)在小儿骨髓增生异常综合征(MDS)中的作用。1976年至2001年,共有94例连续性小儿MDS患者接受了异基因BMT,其中难治性贫血(RA)25例、环形铁粒幼细胞性难治性贫血(RARS)2例、难治性贫血伴原始细胞增多(RAEB)20例、转化中的RAEB(RAEB-T)14例、青少年粒-单核细胞白血病(JMML)32例或慢性粒-单核细胞白血病(CMML)1例。估计3年生存率、无事件生存率(EFS)、非复发死亡率和复发率分别为50%、41%、28%和29%。RA/RARS患者估计3年生存率为74%,而RAEB患者为68%,JMML/CMML患者为33%。多变量分析显示,与RA/RARS和RAEB患者相比,RAEB-T或JMML患者死亡可能性分别高3.9倍和3.7倍(P分别为0.005和0.004)。RAEB-T患者复发可能性高5.5倍(P=0.01)。43例存活患者的中位随访时间为10年(范围1 - 25年)。我们得出结论,小儿MDS患者接受异基因BMT耐受性良好且可能治愈。