Myelodysplastic Syndromes (MDS) and autoimmune disorders (AD): Cause or consequence?

被引：85

作者：

Braun, Thorsten ^{[1
]}

Fenaux, Pierre ^{[1
]}

机构：

[1] Univ Paris 13, Hop Avicenne, Dept Haematol, F-93009 Bobigny, France

来源：

BEST PRACTICE & RESEARCH CLINICAL HAEMATOLOGY | 2013年 / 26卷 / 04期

关键词：

autoimmunity; MDS; AML; CMML; Treg; REGULATORY T-CELLS; NECROSIS-FACTOR-ALPHA; BONE-MARROW FAILURE; NF-KAPPA-B; LOW-RISK; RELAPSING POLYCHONDRITIS; SWEETS-SYNDROME; POLYMYALGIA-RHEUMATICA; AUTOLOGOUS LYMPHOCYTES; INTERFERON-GAMMA;

D O I：

10.1016/j.beha.2013.09.003

中图分类号：

R5 [内科学];

学科分类号：

100201 [内科学];

摘要：

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.

引用

页码：327 / 336

页数：10

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