Department of Medicine and Biosystemic Science, Kyushu University Graduate School of Medical Sciences, Fukuoka.
Department of Internal Medicine and Clinical Immunology, Kyushu University Beppu Hospital, Beppu.
Medicine (Baltimore). 2021 Apr 2;100(13):e25406. doi: 10.1097/MD.0000000000025406.
We evaluated the clinical characteristics of autoimmune manifestations (AIMs) associated with myelodysplastic syndrome (MDS) to elucidate whether AIMs impacted MDS outcomes in Japan.This retrospective study including 61 patients who received a new diagnosis of MDS between January 2008 and December 2015 was conducted by the review of electronic medical records for the presence of AIMs within a 1-year period prior to or following the diagnosis of MDS.AIMs were identified in 12 of the 61 (20.0%) patients with MDS. The neutrophil counts and C-reactive protein levels in peripheral blood were significantly elevated in patients with AIMs, and the survival was shorter in those with AIMs compared to those without AIMs. Multivariate analysis demonstrated that the presence of AIMs and higher-risk disease according to the International Prognositic Scoring System (IPSS) were independent risk factors for increased mortality (hazard ratio, 4.76 and 4.79, respectively).This retrospective study revealed that the prognosis was poor in patients with MDS-associated AIMs. The treatment of MDS using the current algorithms is based on prognostic scoring systems such as IPSS. Treatment strategies for patients with MDS-associated AIMs should be reconsidered, even in those with low-risk MDS according to the IPSS.
我们评估了与骨髓增生异常综合征（MDS）相关的自身免疫表现（AIMs）的临床特征，以阐明 AIMs 是否影响日本 MDS 的预后。这项回顾性研究纳入了 61 例 2008 年 1 月至 2015 年 12 月期间新诊断为 MDS 的患者，通过查阅电子病历，评估 MDS 诊断前或后 1 年内是否存在 AIMs。61 例 MDS 患者中有 12 例（20.0%）存在 AIMs。AIMs 患者的外周血中性粒细胞计数和 C 反应蛋白水平显著升高，且存在 AIMs 的患者生存时间短于无 AIMs 的患者。多因素分析显示，存在 AIMs 和根据国际预后评分系统（IPSS）定义的高危疾病是死亡风险增加的独立危险因素（危险比分别为 4.76 和 4.79）。这项回顾性研究显示，伴发 AIMs 的 MDS 患者预后较差。目前的 MDS 治疗算法基于预后评分系统，如 IPSS。即使根据 IPSS 为低危 MDS 患者，也应重新考虑伴发 AIMs 的 MDS 患者的治疗策略。
