风湿病合并骨髓增生异常综合征的临床分析

The clinical analysis of rheumatic diseases complicated with myelodysplastic syndrome

目的 探讨风湿病与骨髓增生异常的相关性。方法 回顾性分析 76 9例风湿病患者的临床资料 ,收集 7例白塞病 (BD)、2例系统性红斑狼疮 (SLE)、1例成人Still’s病、2例类风湿关节炎 (RA)合并骨髓增生异常综合征 (MDS)共 11例患者的临床资料、血象和骨髓象及部分骨髓活检病理。 7例BD患者中 ,3例合并难治性贫血 (MDS RA) ,2例合并难治性贫血伴原始细胞增多 (MDS RAEB ) ,1例合并转化型难治性贫血伴原始细胞过多 (MDS RAEBt) ,1例合并慢性粒 单细胞白血病 (MDS CMML) ;2例SLE、1例成人Still’s病、2例RA均合并MDS RA。结果 11例均有贫血 ,10例白细胞低下 ,9例血小板减少。 10例骨髓增生活跃 ,1例为低增生型的MDS。 4例BD发病在前 ,3例BD与MDS同时出现 ;1例SLE、1例成人Still’s病、2例RA均在MDS之前发病。结论 风湿病与MDS之间可能有相关性。

Objective To clarify the relationship between Rheumatism( Behcet’s disease,Still’s disease, rheumatoid arthritis and systemic lupus erythematosus) and myelodysplastic syndrome(MDS).Methods The clinical manifestations of 126 patients with BD,20 patients with Still’s disease,526 patients with RA and 97 patients with SLE from Beijing Jishuitan Hospital were analysed retrospectively.12 of them were compatible with the diagnosis of MDS.Results All 12 patients refractory anemia,10 had leucocytopenia,and 9 had thrombocytopenia . 11 of them showed proliferative bone marrow.One showed hypoplastic bone marrow.MDS occurred several years after the rheumatic diseases diagnosis (BD in 4 cases,SLE,RA and Still’s disease);3 BD and MDS occurred simultaneously.Conclusions It is suggested that rheumatic diseases and MDS have a close association and both have immunological abnormalities.