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Topical Collection "Myelodysplastic Syndromes: From Inflammatory to Therapeutic Approaches"
A topical collection in Journal of Clinical Medicine (ISSN 2077-0383). This collection belongs to the section "Hematology".Viewed by 357
Interests: MDS; CMML; Treg; ruxolitinib; AML progression
Topical Collection Information
Impaired function of immune cells and aberrant inflammatory response of the immune system is frequently observed for patients with myelodysplastic syndromes (MDS) and chronic myelomonocytic leukemia (CMML). Increased secretion of inflammatory cytokines, including tumor necrosis factor-(TNF)-α and interferon (IF)-γ, contributes to cell death of myeloid precursor cells and triggers transformation to acute myeloid leukemia (AML). For CMML patients, a phospho-STAT5 hypersensitive response to GM-CSF has been identified that is positively correlated with high-risk disease. Altered function of cytotoxic, regulatory (Treg), helper (Th17) T cells, and natural killer (NK) cells contribute to cytopenias and disease progression. Autoimmune disorders (AD) observed for MDS and CMML patients include vasculitis, seronegative polyarthritis, relapsing polychondritis, or neutrophilic dermatosis. In a subset of patients, cytopenias are the consequence of autoreactive cytotoxic T cells and respond to immunosuppressive treatment (IST). Interestingly, despite IST, other drugs are of interest by treating MDS and CMML-associated AD and inflammatory response as the hypomethylating agent azacytidine improves cytopenias, delays disease progression, and controls AD by sparing corticosteroids and other immunosuppressive drugs. The immunomodulatory drug lenalidomide improves anemia in specific subtypes of MDS and inhibitors of the JAK-STAT pathway should be evaluated for the treatment of selected MDS and CMML patients.
Prof. Dr. Thorsten Braun
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- Autoimmune disorders
- T cells