80例骨髓增生异常综合征患者预后因素分析  

An analysis of prognostic factors in 80 myelodysplastic syndrome

作　　者：许晓倩[1] 王健民[1] 吕书晴[1] 陈莉[1] 杨建民[1] 章卫平[1] 宋献民[1] 许燕群[1] 龚胜蓝[1] 侯军[1] 倪雄[1] 

机构地区：[1]第二军医大学长海医院血液内科,上海200433

出　　处：《中华血液学杂志》2008年第11期723-727,共5页Chinese Journal of Hematology

摘　　要：目的探讨骨髓增生异常综合征（MDS）患者WHO分型、临床及血液学特征，以及MDS患者国际预后积分系统（IPSS）分组特点。方法采用WHO新分型方案对80例MDS患者进行诊断及分型。对确诊的MDS患者的临床表现、血常规结果、骨髓象及转归进行总结性分析。结果MDS患者中位发病年龄为47岁，低于国外学者的报道。染色体异常率为35．14％，检出的异常核型分布以＋8最多，不同于欧、美洲国家和日本的大样本研究结果。74例患者经IPSS分组及追踪观察，11例转化为急性白血病。单变量分析提示年龄、染色体、原始细胞比例及外周血细胞减少系列数对患者有预后意义。IPSS低危组与中危-Ⅰ组生存率差异无统计学意义（P〉0．05），其余各组间差异有统计学意义（P〈0．05）。WHO分型中难治性贫血（RA）、难治性血细胞减少伴多系增生异常（RCMD）与RA伴有原始细胞过多（RAEB）之间生存率差异有统计学意义（P值均〈0．05），RAEB-Ⅰ与RAEB-Ⅱ之间生存率差异有统计学意义（P〈0．05）。结论该组MDS患者的发病年龄、WHO亚型分布、染色体异常检出率及分布与欧、美洲国家和日本不同。WHO分型将RAEB分为RAEB-Ⅰ和RAEB-Ⅱ，对临床诊断、治疗和预后有益。IPSS同样适用于中国人，对选择合适的治疗方案及提示预后具有重要意义。Objective To investigate the WHO classification, clinical and hematological features and risk group of International Prognostic Scoring System （IPSS） in patients with myelodysplastic syndrome （MDS）. Methods The diagnosis and classification of MDS patients were defined according to the WHO classification. The clinical manifestations, bemogram, bone marrow biopsy and prognosis were retrospectively analyzed. Results The median age at diagnosis of MDS was 47 yrs being younger than that in some foreign reports. The frequency of abnormal karyotype was 35.14 % and ＋ 8 was the most frequent abnormal karyotype in our study. Eleven of 74 patients transformed into leukemia. Univariate analysis showed that age, chromosome abnormality, percentage of bone marrow blast cells and number of eytopenias were significantly related to prognosis. There was a statistical difference in cum survival rate between IPSS subcategories （ P 〈 0.05 ） except that between low- and intermediate Ⅰ - risk subcategory （ P 〉 0.05 ）. There were statistical differences for refractory anemia（RA） vs RA with excess blast（ RAEB）, refractory cytopenias with multilineage dysplasia （RCMD） vs RAEB and RAEB- Ⅰ vs RAEB- Ⅱ （P 〈 0. 05）. Conclusions There were differences in age of disease onset, distribution of WHO, sub-classification and abnormal karyotype in this cohort of MDS patients as compared with those in Europe and Japan. It is helpful in diagnosis, treatment and prognosis to divide RAEB into RAEB-Ⅰ and RAEB-Ⅱ. IPSS was well applicable in Chinese MDS patients.

关 键 词：骨髓增生异常综合征 WHO分型 国际预后积分系统 

分 类 号：R686[医药卫生—骨科学]