Myelodysplastic Syndromes (MDS) and autoimmune disorders (AD): Cause or consequence?
Myelodysplastic Syndromes (MDS) and autoimmune disorders (AD): Cause or consequence?
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DOI：
10.1016/j.beha.2013.09.003
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发表时间：
2013-12-01
影响因子：
2.1
通讯作者：
Fenaux, Pierre
中科院分区：
文献类型：
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作者：
Braun, Thorsten;Fenaux, Pierre
骨髓增生异常综合征（MDS）和慢性粒单核细胞白血病（CMML）常与自身免疫性疾病（AD）的临床表现和免疫系统的炎症反应相关。AD伴发MDS和CMML包括血管炎、血清阴性多发性关节炎和嗜酸性皮肤病。罕见AD（包括复发性多囊炎）与MDS密切相关，因为在这些患者中，MDS在病程中被诊断的比例很高。抗核抗体（ANA）常见于无AD临床表现的MDS患者。在一部分患者中，MDS和由此产生的血细胞减少似乎是自身反应性免疫活性的结果，并可能对免疫抑制治疗（IST）有反应。炎性细胞因子如肿瘤坏死因子-（TNF）-α和干扰素（IF）-γ的释放增加触发髓样前体细胞的凋亡，导致血细胞减少。包括细胞毒性、调节性（Treg）、辅助性（Th 17）T细胞和NI（T细胞）在内的免疫细胞的功能受损似乎也预测对IST的应答、AD的结果和发生。(C)2013爱思唯尔有限公司保留所有权利。
Myelodysplastic Syndromes (MDS) and Chronic Myelomonocytic Leukemia (CMML) are frequently associated with clinical manifestations of autoimmune disorders (AD) and inflammatory response of the immune system. AD accompanying MDS and CMML include vasculitis, seronegative polyarthritis and neutrophilic dermatosis. Rare AD including relapsing polychondritis is strongly associated with MDS as in a high proportion of those patients MDS is diagnosed during disease course. Antinuclear antibodies (ANA) are frequently found among MDS patients without clinical manifestation of AD. In a subset of patients, MDS and resulting cytopenias appear to be the consequence of auto reactive immunologic activity and may respond to immunosuppressive treatment (IST). Increased release of inflammatory cytokines like tumor necrosis factor-(TNF)-alpha and interferon (IF)-gamma triggers apoptosis of myeloid precursor cells leading to cytopenias. Impaired function of immune cells including cytotoxic, regulatory (Treg), helper (Th17) T cells and NI( cells also appears to predict response to IST, outcome and occurrence of AD. (C) 2013 Elsevier Ltd. All rights reserved.