title: Myelodysplastic Syndromes (MDS) and autoimmune disorders (AD): cause or consequence?
abstract: 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)-α and interferon (IF)-γ triggers apoptosis of myeloid precursor cells leading to cytopenias. Impaired function of immune cells including cytotoxic, regulatory (Treg), helper (Th17) T cells and NK cells also appears to predict response to IST, outcome and occurrence of AD.
authors: Department of Haematology, Hôpital Avicenne, University Paris 13, Bobigny, France.
authors: Thorsten
authors: T
authors: Braun
authors: Department of Haematology, Hôpital Avicenne, University Paris 13, Bobigny, France. Electronic address: pierre.fenaux@avc.aphp.fr.
authors: Pierre
authors: P
authors: Fenaux
mesh_terms: D000974:Antibodies, Antinuclear / Q000097:blood / Q000276:immunology; D001327:Autoimmune Diseases / Q000097:blood / Q000150:complications / Q000175:diagnosis / Q000276:immunology / Q000473:pathology / Q000628:therapy; D006801:Humans; D007165:Immunosuppression Therapy; D007371:Interferon-gamma / Q000097:blood / Q000276:immunology; D007694:Killer Cells, Natural / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D009190:Myelodysplastic Syndromes / Q000097:blood / Q000150:complications / Q000175:diagnosis / Q000276:immunology / Q000473:pathology / Q000628:therapy; D023461:Myeloid Progenitor Cells / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D050378:T-Lymphocytes, Regulatory / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D058504:Th17 Cells / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D014409:Tumor Necrosis Factor-alpha / Q000097:blood / Q000276:immunology
chemical_list: D000974:Antibodies, Antinuclear; C573415:IFNG protein, human; D014409:Tumor Necrosis Factor-alpha; D007371:Interferon-gamma
keywords: AML; CMML; MDS; Treg; autoimmunity