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