原发性骨髓增生异常综合征合并自身免疫异常患者临床特征及生存研究

Clinical characteristics and survival research in patients with myelodysplastic syndrome combined with autoimmune anomaly

目的观察原发性骨髓增生异常综合征（myelodysplastic syndrome ,MDS）合并自身免疫异常患者的临床特征，探讨合并自身免疫异常对MDS患者转归及生存期的影响。方法分析2009年1月～2012年6月在河北省石家庄市赵县妇幼保健院进行治疗的146例MDS患者的临床资料，根据是否合并自身免疫异常将其分为合并自身免疫异常组（n=31）和免疫学检查正常组（n：115），比较其一般情况、合并自身免疫性疾病情况、血常规指标、染色体核型分布以及转向率、中位生存时间等指标。结果146例MDS患者中，15例（10．27％）合并自身免疫性疾病，另有16例（10．96％）患者出现免疫学检查异常而无临床症状，二者合计31例，占比（21．23％）。合并自身免疫异常组患者〈60岁者比例、女性比例明显高于免疫学检查正常组，差异具有统计学意义（P〈0．05）。两组患者外周血血常规检查指标、染色体核型分布、转白率等指标无差异（P〉0．05）。合并自身免疫异常组患者中位生存期明显短于免疫学检查正常组，差异具有统计学意义（P〈0．05）。结论MDS患者合并自身免疫异常多见于年龄〈60岁的女性，且合并自身免疫异常的MDS患者中位生存期缩短．提示自身免疫异常对MDS的预后可能存在不良影响。

Objective To observe the clinical characteristics in patients with myelodysplastic syndrome combined with autoimmune anomaly, and to investigate the influence of prognosis and survival time in patients with myelodysplastic syndrome combined with autoimmune anomaly. Methods A total of 146 cases of clinical data from January 2009 to June 2012 were analyzed, all the patients were divided into autoimmune anomaly group （n = 31） and immune normal group （n = 115）; general condition, autoimmune disease conditions, blood routine index, chromosome karyotype distri- bution, rate of turn leukemia and median survival time were compared. Results Of all the 146 cases, 15 patients （10.27%） of MDS were found having autoimmune anomaly and 16 cases （10.96%） of MDS were found having immunological tests anomaly without clinical symptoms, the two situations were added together for 31 cases （21.23%）. The percentage of less than 60 years old and the female in autoimmune anomaly group were higher than those in immune normal group, the differences were statistically significant （P 〈 0.05）. The differences of blood routine index, chromosome karyotype distribution and rate of turn leukemia were not statistically significant （P 〉 0.05）; median survival time in autoimmune anomaly group were shorter than that in immune normal group, the differences were statistically significant （P 〈 0.05）. Conclusion The female patients in the age of less than 60 years old with autoimmune anomaly take the main percentage in patients with MDS; median survival time in patients with MDS and autoimmune anomaly is shorter, it point out that the autoimmune anomaly may take a harmful effect to the prognosis of MDS.