骨髓增生异常综合征合并自身免疫性疾病的临床特征及预后分析

Clinical Features and Survival Analysis in Myelodysplastic Syndromes with Autoimmune Diseases

摘要: 目的：探讨伴发自身免疫性疾病(AIDs)的骨髓增生异常综合征(MDS)患者的临床特征并分析其对疾病预后的影响。方法：回顾性分析206例初诊MDS患者的临床资料，依据有无合并AIDs分为两组，比较合并AIDs组与未合并组间患者的一般临床特征及预后。结果：在206例MDS患者中，共57例(27.7%)合并自身免疫病，其中血管炎最常见。合并AIDs组患者女性占比高，在2016 WHO分型中MDS伴多系血细胞发育异常型(MDS-MLD型)最常见，在国际预后积分系统(IPSS)分级中多为低危级和中危-1级；合并AIDs组与未合并组患者的无疾病进展生存时间(PFS)分别为18.6个月vs 12.4个月(p = 0.010)，中位总生存时间(OS)分别为40个月vs 28.8个月(p = 0.043)。结论：合并自身免疫性疾病的MDS患者女性比例较高，分型及分期上以中低危为主，多有较长的PFS及OS。

Abstract: Objective: To explore the impact of myelodysplastic syndrome (MDS) patients with autoimmune diseases (AIDs) on clinical features and prognosis. Methods: The clinical data from 206 newly diagnosed MDS patients were retrospectively analyzed. All patients were classified as with and without AIDs. Clinical characteristics and outcomes between both groups were compared. Results: Among 206 MDS patients, 57 cases (27.7%) are with AIDs and vasculitis is the most common AIDs. MDS patients with AIDs were more common in females, mainly associated with MDS with multilineage dysplasia (MDS-MLD) among the WHO categories, and tending to the lower categories (low and intermediate-1 risk group) in International Prognostic Scoring Systems (IPSS). Median progression-free survival (PFS) was 18.6 months for patients with AIDs versus 12.4 months for those without (log-rank test, P = 0.010), while the median overall survival (OS) was 40 months versus 28.8 months (P = 0.043). Conclusion: MDS patients with AIDs are more frequent among female, with a lower risk among the WHO categories and IPSS, and most of them have longer PFS and OS.