中文摘要：

目的评价WPSS预后积分系统在我国骨髓增生异常综合征（MDS）患者预后判断中的作用，并对常规实验室检查指标用于MDS患者预后判断进行初步研究。方法对染色体核型结果可供分析的164例成人原发MDS患者进行回顾性分析。结果164例患者中位随访时间19（1—138）个月，中位生存（MS）期36个月，2年预期生存（PS）率60％，5年PS率42％。按WPSS评分，极低危组、低危组、中危组、高危组和极高危组的2年PS率分别为100％、96％、81％、38％和14％，5年PS率分别为100％、83％、54％、20％和0，Log—rank检验各组总体生存（OS）率差异有统计学意义（P〈0．01）。常规实验室检查中，Hb≥80g/L、平均红细胞体积（MCV）升高（〉95fl）、平均红细胞血红蛋白含量（MCH）升高（〉32pg）、BPC≥100×10^9／L的患者预后较好；骨髓原始细胞比例（Blast）≥0．05的患者较〈0．05的患者预后差，骨髓细胞发育异常≥2系的患者预后较差；细胞组化检查中性粒细胞碱性磷酸酶（N-ALP）阳性指数升高（〉173．21）、有核红细胞糖原染色（E—PAS）阴性的患者预后较好，小巨核酶标染色可见淋巴样小巨核细胞（MEGly）的患者预后较差。另外，血清乳酸脱氢酶水平升高（≥300U／L）的患者预后较差。将上述单因素分析有预后意义的参数纳入COX多因素模型筛选，MCV、MEGly、Blast具有独立预后意义（P值分别为0．011、0．013、0．016）。选取MCV、MEGly及WHO分型提出改良的WPSS积分系统，低危组、中危组、高危组患者的2年PS率分别为94％、68％和49％，5年PS率分别为86％、53％和14％，经Log—rank检验各组OS率差异有统计学意义（P〈0．01）。结论与WHO分型相适应的WPSS适合用于判断我国MDS患者的预后。采用常规实验室检查指标中的MCV、MEGly及WHO分型提出的改良WPSS积分系统可望提供适于我国现阶段基层单位?

英文摘要：

Objective To investigate the prognostic value of a modified WPSS based on routine laboratory parameters in Chinese patients with myelodysplastic syndromes （MDS）. Methods One hundred and sixty four adult MDS patients were retrospectively analyzed. Results The median follow-up time was 19 （ 1 - 138 ） months and the median survival（MS） was 36 months. 2-year prospective survival（PS） was 60% and 5- year PS was 42%. In patients with very low-risk, low-risk, intermediate-risk, high-risk and very high-risk stratified by WPSS, 2-year PS was 100% , 96% , 81% , 38% and 14% , and 5-year PS was 100% , 83% , 54%, 20% and 0, respectively （ P 〈 0.01 ）. Among parameters of laboratory routine examination, elevated mean cell volume （MCV） , mean cell hemoglobin （MCH） , neutrophil alkaline phosphatase （N-ALP） index and nucleated RBC PAS negative were good prognostic factors, while reduced Hb, BPC and bone marrow elevated blasts, dysplasia more than 1 lineage, and lymphocyte-like micromegakaryocyte （ MEGly ） as well aselevated serum LDH were poor prognostic factors in uni-variable analysis. Among them, MCV, MEGly and blast had independent prognostic significance in multi-variable analysis （P = 0. 011, 0. 013 and 0. 016, respectively）. WPSS was modified by omitting chromosomal karyotype and ,transfusion dependence and adding MCV and MEGly. In patients with low-risk, intermediate-risk and high-risk stratified by modified WPSS, 2- year PS was 94% , 68% and 49% , respectively; and 5-year PS was 86% , 53% and 14% , respectively （ P 〈 0.01 ）. Conclusion The modified WPSS worked well for prognostic prediction in Chinese patients with MDS.