Fudan-Cinpathogen Clinical and Molecular Research Center (CMRC), Institute of Biomedical Sciences, Fudan University, Shanghai, China.
Huashan Hospital, Fudan University, Shanghai, China.
Int J Hematol. 2009 Oct;90(3):361-369. doi: 10.1007/s12185-009-0403-5. Epub 2009 Aug 29.
We characterized the prevalence, clinical and cytogenetic characteristics and survival of 435 patients diagnosed with de novo MDS in a single laboratory according to WHO criteria, and compared the utility of different scoring systems to predict survival for individual subtypes of MDS. The mean follow-up period was 25.1 (5.5-53.2) months. Our results confirm major differences in the age-distribution and prevalence of individual subtypes of MDS between Asian and Western patients with a median age of 58 years and a predominance of RCMD (69.9%). Survival rates were similar to those reported in the West: the 3-year survival rate for MDS was 46.7% with a median survival time for RCMD of 38 months and RAEB, 10 months. We found that the IPSS and WPSS scoring systems, which are weighted heavily by blast cell count and karyotype, were not independent predictors for survival in RCMD patients. Multivariate analysis demonstrated that a scoring system based on age (> or =60 years), ANC (<1.0 x 10(9)/L), Hb (<90 g/L), number of cytopenias and complex karyotype is a more useful predictor of survival in RCMD.
我们根据世界卫生组织（WHO）标准，对一家实验室诊断的 435 例初诊骨髓增生异常综合征（MDS）患者的流行率、临床和细胞遗传学特征及生存情况进行了分析，并比较了不同评分系统对 MDS 各亚型患者生存预测的效用。中位随访时间为 25.1（5.5-53.2）个月。我们的研究结果证实，亚洲和西方 MDS 患者的年龄分布和各亚型的流行率存在显著差异，中位年龄为 58 岁，以难治性细胞减少性贫血（RCMD）（69.9%）多见。生存率与西方报道的相似：MDS 的 3 年生存率为 46.7%，RCMD 的中位生存时间为 38 个月，RAEB 为 10 个月。我们发现，IPSS 和 WPSS 评分系统（严重依赖于原始细胞计数和核型）对 RCMD 患者的生存不是独立的预测因素。多变量分析显示，基于年龄（≥60 岁）、ANC（＜1.0×10(9)/L）、Hb（＜90g/L）、血细胞减少数量和复杂核型的评分系统，是 RCMD 患者生存的更有用的预测指标。
