风湿性疾病合并骨髓增生异常综合征九例临床分析

Rheumatic diseases associated with myelodysplastic syndromes:nine cases report

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 investigate rheumatic disease manifestations in patients with myelodysplastic syndromes and explore possible causes. Methods Nine myelodysplastic syndromes （MDS） inpatients with rheumatic disease manifestations were reviewed retrospectively. Results Nine patients with the diagnosis of MDS had active rheumatic disease manifestations with various degree of hypergammaglobulinemia and positive autoantibodies, Two patients had rheumatoid arthritis （RA） and RA＋anti-phospholipid syndrome （APS）, four systemic lupus erythematosus （SLE） and SLE＋polymyositis （PM）, one patients had adult onset Still＇s disease, one with acute arthritis and cutaneous vassulitis, one had lupus-like manifestations including polyserositis, albuminuria, hypocomplementemia and positive Commb＇s test, Conclusion The rheumatic disease manifestations in patients with myelodysplastic syndromes are characterized by various active rheumatic disease manifestation. Immunological abnormalities and ineffective hematopoiesis are persistent. Immunologic abnormalities in MDS such as impaired function of T and B cells, hypergammaglobulinemia, positive autoantihodies, may he the causes of various rheumatic manifestations. We suggest that there is a significant association between myelodysplastic syndrome and rheumatic diseases.