Anderson J E, Appelbaum F R, Storb R
Clinical Research Division, Fred Hutchinson Cancer Research Center, Seattle, WA 98104, USA.
Leuk Lymphoma. 1995 Mar;17(1-2):95-9. doi: 10.3109/10428199509051708.
Ninety-three patients with myelodysplastic syndrome with either excess blasts or life-threatening cytopenia received cyclophosphamide and total body irradiation (88 patients) or busulfan (5 patients) followed by allogeneic (90 patients) or syngeneic (3 patients) marrow infusion. Thirty-eight patients are disease-free survivors between 1.8 and 11.3 years (median 6.1 years) after transplantation. The median Karnofsky performance status of the survivors is 100%, and only 2 have a performance status of < 80%. Eighteen patients relapsed and the remaining 37 died without evidence for relapse. The 5-year actuarial disease-free survival, relapse, and non-relapse mortality rates are 40%, 29%, and 44%, respectively. The actuarial relapse rate was higher among patients with excess blasts at the time of transplantation compared to those without excess blasts (49% vs. 4%, p = 0.0001), resulting in a lower disease-free survival (31% vs. 54%, p = 0.07). Increasing age was associated with a lower disease-free survival (p = 0.02). In a multivariate analysis, younger age, shorter disease duration, and absence of excess blasts were associated with improved outcome. We recommend that patients with myelodysplasia with appropriate marrow donors, especially patients less than age 40, undergo marrow transplantation early during the disease course, before the disease progresses or life-threatening complications develop.
93例伴有原始细胞增多或危及生命的血细胞减少的骨髓增生异常综合征患者接受了环磷酰胺和全身照射(88例患者)或白消安(5例患者)治疗,随后接受了异基因(90例患者)或同基因(3例患者)骨髓输注。38例患者在移植后1.8至11.3年(中位时间6.1年)为无病生存者。生存者的中位卡氏功能状态评分为100%,只有2例患者的功能状态评分<80%。18例患者复发,其余37例死亡,无复发证据。5年精算无病生存率、复发率和非复发死亡率分别为40%、29%和44%。与移植时无原始细胞增多的患者相比,移植时伴有原始细胞增多的患者精算复发率更高(49%对4%,p = 0.0001),导致无病生存率更低(31%对54%,p = 0.07)。年龄增加与无病生存率降低相关(p = 0.02)。多因素分析显示,年龄较小、病程较短和无原始细胞增多与预后改善相关。我们建议,有合适骨髓供者的骨髓增生异常综合征患者,尤其是年龄小于40岁的患者,在病程早期、疾病进展或出现危及生命的并发症之前接受骨髓移植。