Wang Xiao-Qin
Hematology Department of Huashan Hospital, Fudan University, Shanghai 200040, China.
Zhonghua Nei Ke Za Zhi. 2009 Aug;48(8):633-7.
To investigate the prognostic factors of myelodysplastic syndrome (MDS) in cases diagnosed with WHO classification and evaluate the validity of International Prognostic Scoring System (IPSS) and WHO Classification-Based Prognostic Scoring System (WPSS) so as to establish a new score system for Chinese MDS patients.
Four hundred and thirty-five patients diagnosed as primary MDS in Sino-US Leukemia Cooperative Group of Shanghai were studied prospectively to identify the prognostic factors by Log-rank test and Cox regression model.
The 3-year survival rate of MDS was 46.7%. The median survival time of refractory cytopenia with multilineage dysplasia (RCMD) subtype was 38 months and that of refractory anemia with excess blasts (RAEB) subtype was 10 months. Four hundred and twenty-four patients have successful karyotyping. According to the IPSS score, low risk, intermediate risk- I, intermediate risk-II and high risk group accounted for 38/424 (9.0%), 282/424 (66.5%), 74/ 424 (17.5%) and 30/424 (7.1%). Multivariate analysis of Cox model showed old age, lower neutrophil absolute count (NAC), lower hemoglobin, lower platelet count and IPSS group were independent factors associated with overall survival time (OS). WPSS was not an independent prognostic factor. For cases with RCMD, platelet count, IPSS score, IPSS cytogenetics group and chromosome abnormality were not independent prognostic factors; whereas age (> or = 60 years old), NAC (< 1.0 x 10(9)/L), Hb (< 90 g/L) and degree of cytopenia were independent factors. A new score system was established based on these factors. It had better ability to predict OS for RCMD patients as it was proved by univariate and multivariate analysis.
The survival rate and median survival time of MDS in Chinese are similar to those in Western. IPSS score can predict prognosis for all MDS patients and it is better than WPSS. However, both of them have no validity for the prognostication of RCMD subtype. A new score system which can distinguish the different prognoses for RCMD patients is suggested.
探讨按世界卫生组织（WHO）分类诊断的骨髓增生异常综合征（MDS）的预后因素，评估国际预后评分系统（IPSS）和基于WHO分类的预后评分系统（WPSS）的有效性，从而为中国MDS患者建立一个新的评分系统。
对上海中美白血病协作组诊断为原发性MDS的435例患者进行前瞻性研究，通过对数秩检验和Cox回归模型确定预后因素。
MDS患者的3年生存率为46.7%。多系发育异常的难治性血细胞减少症（RCMD）亚型的中位生存时间为38个月，原始细胞增多的难治性贫血（RAEB）亚型的中位生存时间为10个月。424例患者成功进行了核型分析。根据IPSS评分，低危、中危-I、中危-II和高危组分别占38/424（9.0%）、282/424（66.5%）、74/424（17.5%）和30/424（7.1%）。Cox模型多因素分析显示，年龄、中性粒细胞绝对计数（NAC）降低、血红蛋白降低、血小板计数降低和IPSS分组是与总生存时间（OS）相关的独立因素。WPSS不是独立的预后因素。对于RCMD病例，血小板计数、IPSS评分、IPSS细胞遗传学分组和染色体异常不是独立的预后因素；而年龄（≥60岁）、NAC（<1.0×10⁹/L）、血红蛋白（<90g/L）和血细胞减少程度是独立因素。基于这些因素建立了一个新的评分系统。单因素和多因素分析证明，它对RCMD患者的OS有更好的预测能力。
中国MDS患者的生存率和中位生存时间与西方相似。IPSS评分可预测所有MDS患者的预后，且优于WPSS。然而，两者对RCMD亚型的预后评估均无效。建议建立一个能区分RCMD患者不同预后的新评分系统。
