Autoimmune disorders are common in myelodysplastic syndrome patients and confer an adverse impact on outcomes
Autoimmune disorders are common in myelodysplastic syndrome patients and confer an adverse impact on outcomes
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DOI：
10.1007/s00277-018-3302-0
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发表时间：
2018-08-01
影响因子：
3.5
通讯作者：
Valcarcel, David
中科院分区：
文献类型：
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作者：
Montoro, Julia;Gallur, Laura;Valcarcel, David
骨髓增生异常综合征 (MDS) 或慢性粒单核细胞白血病 (CMML) 患者同时存在自身免疫性疾病 (AD) 已被广泛认识到，尽管关于其患病率和对潜在血液学过程结果的影响有不同的结果。本研究旨在分析 142 名诊断为 MDS 和 CMML 的患者中 MDS 合并 AD 的患病率、临床特征和结果。 AD 通过临床症状的存在或相容的血清学检测来确定。总共有 48% 的患者被诊断患有 AD，其中甲状腺功能减退症是最常见的临床 AD 报告 (8%)，抗核抗体是最常见的血清学参数 (23.2%)。 AD 的存在与女性、较低的血红蛋白水平和较高的 IPSS-R 相关。 AD 患者的总生存率低于非 AD 患者（30 个月时为 69% vs. 88%；HR 2.75，P = 0.008）。值得注意的是，临床而非孤立的免疫血清学参数对 AD 患者的预后有影响。最后，在多变量分析中，AD 的存在（HR 2.26）以及疾病风险类别（极低和低与中、高和极高 IPSS-R；HR 4.62）保留了其独立的预后价值（P < 0.001）。总之，AD 在 MDS 和 CMML 患者中普遍存在，并且具有预后意义，特别是在低风险 MDS 患者中。
The coexistence of autoimmune disorders (AD) in patients with myelodysplastic syndrome (MDS) or chronic myelomonocytic leukemia (CMML) has been widely recognized, although with distinct results regarding their prevalence and impact on the outcomes of the underlying hematological process. This study was aimed to analyze the prevalence, clinical characteristics, and outcomes of MDS with AD in a series of 142 patients diagnosed with MDS and CMML. AD was ascertained by both the presence of clinical symptoms or compatible serological tests. In total, 48% patients were diagnosed as having AD, being hypothyroidism the most commonly reported clinical AD (8%) and antinuclear antibodies the most frequent serological parameter identified (23.2%). The presence of AD was associated with female gender, lower hemoglobin levels, and higher IPSS-R. Overall survival for patients with AD was inferior to those with no AD (69 vs. 88% at 30 months; HR 2.75, P = 0.008). Notably, clinical but not isolated immune serological parameters had an impact on the outcomes of patients with AD. Finally, in a multivariate analysis, the presence of AD (HR 2.26) along with disease risk categories (very low and low vs. intermediate, high, and very high IPSS-R; HR 4.62) retained their independent prognostic value (P < 0.001). In conclusion, AD are prevalent in MDS and CMML patients and have prognostic implications, especially in lower-risk MDS patients.