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目的 探讨骨髓增生异常综合征(M D S)与自身免疫性疾病的相关性及其临床意义。方法 回顾分析 7 例伴自身免疫性疾病的 M D S 的临床及实验特点。结果 7 例伴发的自身免疫性疾病中,白塞病 2 例,类风湿性关节炎、C rohn’s病、抗 D 抗体阳性的输血性溶血性贫血、Sw eet’s综合征及坏疽性脓皮病各 1 例。3 例自身免疫性疾病发生于 M D S 之前,1 例与 M D S 同时发生,3 例发生于 M D S 之后。7 例患者经糖皮质激素及免疫抑制剂治疗后,自身免疫性疾病均得以明显缓解,但M D S 预后仍较差,其中 2 例在半年内转变为急性髓细胞白血病,化疗后均未缓解而死亡,3 例死于 R A 期,其他正在治疗中。结论 部分 M D S 与自身免疫性疾病有密切联系,伴自身免疫性疾病是 M D S 预后不良的因素。
Objective To investigate the relationship between myelodysplastic syndrome (MDS) and autoimmune diseases and its clinical significance. Methods The clinical and experimental characteristics of 7 patients with autoimmune diseases were retrospectively analyzed. Results Among the 7 cases of autoimmune diseases associated with Behcet’s disease, 2 were Behcet’s disease, Rheumatoid arthritis, Crohn’s disease, anti-D antibody-positive transfusion hemolytic anemia, Swet’s syndrome and pyoderma gangrenosum 1 case. Three autoimmune diseases occurred before M D S, one with M D S and three with M D S. Seven patients were treated with glucocorticoids and immunosuppressive agents, autoimmune diseases were significantly alleviated, but the prognosis of MDS is still poor, of which two cases of acute myeloid leukemia within six months after chemotherapy did not remission and died , 3 died of RA, others were under treatment. Conclusions Some MDSs are closely related to autoimmune diseases, and autoimmune diseases are the factors of poor prognosis of MDS.