骨髓增生异常综合征合并自身免疫病的WPSS分析  

WPSS analysis on the myelodysplastic syndrome accompanied by autoimmune disease

作　　者：易彦[1] 葛圣雷[2] 

机构地区：[1]中南大学湘雅二医院血液科,湖南长沙410011 [2]中南大学湘雅二医院耳鼻喉科,湖南长沙410011

出　　处：《中国现代医学杂志》2010年第13期2034-2037,共4页China Journal of Modern Medicine

基　　金：国家自然科学基金(No:30700940);湖南省自然科学基金(No:06JJ3011)

摘　　要：目的探讨骨髓增生异常综合征(myelodysplastic syndrome,MDS)合并自身免疫病(autoimmune disease)患者的WPSS积分与临床特点及预后的关系。方法对该院收治的23例合并自身免疫病的MDS患者进行临床回顾性分析和随诊观察,并与同时期收治的52例不合并自身免疫病的普通MDS患者进行比较,分析其临床特点、WPSS积分及预后差异。结果合并自身免疫病的MDS患者与普通MDS患者比较,两组患者中转化为白血病者的比率无显著性差异,但合并自身免疫病MDS患者的生存时间较短。WPSS分析提示,合并自身免疫病的MDS患者中,低危和极低危患者比例较低,而高危和极高危患者比例较高。如WPSS评分相同,合并自身免疫病的MDS患者与普通MDS患者的生存时间无显著性差异。结论合并自身免疫病MDS患者的生存时间较普通MDS患者短,其原因可能是合并自身免疫病MDS患者中,高危和极高危患者比例较高,而低危和极低危患者比例较低。合并自身免疫病的MDS具有高度异质性,WPSS是判断其预后的良好指标。[Objective] To explore the relationship between the World Health Organization classification-based prognostic scoring system （WPSS） scores and the clinical manifestation and prognosis of the myelodysplastie syndromes （MDS） patients with or without autoimmune diseases. [Methods] Twenty-three MDS patients with autoimmune disease and fifty-two MDS patients without autoimmune disease were enrolled and followed up. The clinical manifestation, WPSS scores and prognosis were compared and analyzed retrospectively. [ Results ] Compared with the common MDS patients, the leukemic transformation rate of MDS with autoimmune disease patients did not increased, but the median survival time was shorter. According to WPSS score analysis, in MDS with autoimmune disease patients, the proportion of low/very low risk were relatively smaller, while the proportion of high/very high risk were larger. When WPSS score was the same, there was no significant difference of survival time between the two groups. [Conclusion] Comparing with common MDS patients, the relatively shorter median survival time of MDS with autoimmane disease patients was related to their higher risk proportion. Patients of MDS with autoimmune disease was a highly heterogeneous group. WPSS score might be a powerful predictor of prognosis for MDS with autoimmune disease patients.

关 键 词：骨髓增生异常综合征 自身免疫病 以WHO分类为基础的预后积分系统 

分 类 号：R551.3[医药卫生—血液循环系统疾病]