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目的了解风湿性疾病合并骨髓增生异常综合征(MDS)的临床特点及可能的发病机制。方法对住院确诊的9例风湿性疾病合并MDS的临床特征进行统计分析。结果9例MDS患者中有4例诊断MDS前已有风湿病史,9例确诊MDS时均有活动期风湿病表现,伴有红细胞沉降率增快、不同程度血清免疫球蛋白升高(主要为IgG和IgM)及多种不同的自身抗体阳性,其中2例合并类风湿关节炎(RA)和RA+抗磷脂综合征(APS),4例系统性红斑狼疮(SLE)和SLE+多发性肌炎(PM),1例成人斯蒂尔病,1例伴有急性关节炎、皮肤血管炎等风湿病表现,1例伴有多浆膜腔积液、蛋白尿、血清补体下降、Commb’s试验阳性等狼疮样表现。结论风湿性疾病合并MDS时多有不同的活动期风湿病表现,患者同时存在免疫功能紊乱和造血功能异常,可能由于免疫功能失调。如T、B细胞功能异常、高丙种球蛋白血症、出现自身抗体等引起不同的风湿性疾病表现,说明MDS与风湿性疾病之间存在内在的关联。
Objective To understand the clinical features and possible pathogenesis of rheumatic diseases complicated with myelodysplastic syndrome (MDS). Methods The clinical features of 9 rheumatic diseases diagnosed in hospital were analyzed statistically. Results Four of the nine patients with MDS had a history of rheumatic disease before diagnosis, nine patients with active rheumatoid disease at the time of definite diagnosis of MDS. The rate of erythrocyte sedimentation was increased and serum immunoglobulin was increased (mainly IgG And IgM) and a variety of different autoantibodies, of which 2 were associated with rheumatoid arthritis (RA) and RA + antiphospholipid syndrome (APS), 4 systemic lupus erythematosus (SLE) and SLE + polymyositis ), 1 case of adult stellera disease, 1 case of rheumatic disease accompanied by acute arthritis and cutaneous vasculitis, 1 case of multiple serosal effusion, proteinuria, decreased serum complement, and positive Commb’s test which performed. Conclusion Rheumatic diseases combined with MDS have more active rheumatism performance, patients with immune dysfunction and hematopoietic dysfunction, may be due to immune dysfunction. Such as T, B cell dysfunction, hypergammaglobulinemia, autoantibodies and other rheumatic diseases caused by different performance, indicating that there is an inherent relationship between MDS and rheumatic diseases.