Autoimmunity and Inflammation in Myelodysplastic Syndromes.

摘要: Autoimmune and inflammatory conditions (AICs) are encountered in up to 25% of patients with myelodysplastic syndromes (MDS). A wide range AICs have been reported association MDS can from limited clinical manifestations systemic diseases affecting multiple organs. Vasculitides, connective tissue diseases, arthritis frequently different studies; noninfectious fever constitutional symptoms at presentation common. Associations between specific characteristics vary by study, but the available data suggest that cluster more often younger higher-risk MDS. In general, do not seem confer worse survival, although certain may be associated adverse outcome (e.g. vasculitis) or progression (Sweet's syndrome). Nonetheless, these complications a significant impact on quality life affect timing type MDS-directed therapy. The mainstay management short term relies immunosuppressive drugs. Increasing evidence suggests hypomethylating agents effective treating reduce steroid dependence. While pathogenesis is incompletely understood, growing appreciation cellular immune deregulation, cytokine hypersecretion, genetic heterogeneity underlying improve our understanding common pathways linking MDS, inflammation, autoimmunity.