Certain Autoimmune Manifestations Are Associated With Distinctive Karyotypes and Outcomes in Patients With Myelodysplastic Syndrome A Retrospective Cohort Study
Certain Autoimmune Manifestations Are Associated With Distinctive Karyotypes and Outcomes in Patients With Myelodysplastic Syndrome A Retrospective Cohort Study
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DOI：
10.1097/md.0000000000003091
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发表时间：
2016-03-01
期刊：
影响因子：
1.6
通讯作者：
Yoon, Sung-Soo
中科院分区：
文献类型：
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作者：
Lee, Sang Jin;Park, Jin Kyun;Yoon, Sung-Soo
自身免疫表现（AIM）在骨髓增生异常综合征（MDS）患者中很常见。本研究旨在调查AIM是否与MDS患者的特定细胞遗传学异常和较差的生存率相关。共有67名患有AIM的MDS患者和134名年龄和性别匹配的无AIM的MDS患者入组，所有这些患者均于2000年1月至2014年7月在首尔国立大学医院接受治疗。检查了临床特征、染色体异常和结果。在调整年龄、性别和国际预后评分系统后，评估了 AIM 对死亡率的影响。MDS 诊断时的平均年龄 (+/- SD) 为 54.5 +/- 17.1 岁，44.8% 的患者为男性。中性粒细胞性皮肤病（ND；Sweet 综合征和坏疽性脓皮病）是最常见的 AIM (n=24·36%])，其次是白塞氏病 (10 [15%])、类风湿性关节炎 (9 [13%])、血管炎 (8 [12%])、肌炎 (3 [4%])、脊柱关节病 (3 [4%]) 和系统性关节炎红斑狼疮 (2 [3%])。 ND 和血管炎发生在 MDS 诊断时，而其他 AIM 发生在 MDS 诊断后数年。 5q 缺失与 ND 相关（P=0.001），而 8 三体性与白塞病相关（P=0.015）。引人注目的是，ND 与死亡率增加 1.8 倍相关（95% CI 1.033-3.093；P=0.038）。MDS 患者的某些 AIM 与独特的核型和较差的生存率相关。需要进行更大规模的研究来确认 AIM 的存在是否会影响 MDS 的疾病结果。
Autoimmune manifestations (AIMs) are common in patients with myelodysplastic syndrome (MDS). This study aimed to investigate whether AIMs are associated with a specific cytogenetic abnormalities and worse survival in patients with MDS.A total of 67 MDS patients with AIMs and 134 age- and sex-matched MDS patients without AIMs, all of whom received medical care at Seoul National University Hospital from January 2000 through July 2014, were enrolled. The clinical features, chromosomal abnormalities, and outcomes were examined. The effect of AIMs on mortality was estimated after adjusting for age, sex, and the International Prognostic Scoring System.The mean age (+/- SD) at the time of MDS diagnosis was 54.5 +/- 17.1 years, and 44.8% of patients were male. Neutrophilic dermatosis (ND; Sweet syndrome and pyoderma gangrenosum) was the most prevalent AIM (n=24 36%]), followed by Behcet disease (10 [15%]), rheumatoid arthritis (9 [13%]), vasculitis (8 [12%]), myositis (3 [4%]), spondyloarthropathy (3 [4%]), and systemic lupus erythematous (2 [3%]). ND and vasculitis occurred at the time of MDS diagnosis, whereas other AIMs occurred years after MDS diagnosis. Deletion of 5q was associated with ND (P=0.001), whereas trisomy 8 was associated with Behcet disease (P=0.015). Strikingly, ND was associated with a 1.8-fold increase in mortality (95% CI 1.033-3.093; P=0.038).Certain AIMs in MDS patients are associated with distinctive karyotypes and worse survival. A larger study is needed to confirm whether the presence of AIMs influences disease outcome in MDS.