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Two myeloid neoplasms defined by the presence of RS, include myelodysplastic syndromes with RS (MDS\u2010RS) and MDS\/myeloproliferative neoplasm with RS and thrombocytosis (MDS\/MPN\u2010RS\u2010T).<\/jats:p><\/jats:sec><jats:sec><jats:title>Diagnosis<\/jats:title><jats:p>MDS\u2010RS is a lower risk MDS, with single or multilineage dysplasia (MDS\u2010RS\u2010SLD\/MLD), &lt;5% bone marrow (BM) blasts, &lt;1% peripheral blood blasts and \u226515% BM RS (\u22655% in the presence of <jats:italic>SF3B1<\/jats:italic> mutations). MDS\/MPN\u2010RS\u2010T, now a formal entity in the MDS\/MPN overlap syndromes, has diagnostic features of MDS\u2010RS\u2010SLD, along with a platelet count \u2265450\u2009\u00d7\u200910<jats:sup>9<\/jats:sup>\/L and large atypical megakaryocytes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Mutations and Karyotype<\/jats:title><jats:p>Mutations in <jats:italic>SF3B1<\/jats:italic> are seen in \u226580% of patients with MDS\u2010RS\u2010SLD and MDS\/MPN\u2010RS\u2010T, and strongly correlate with the presence of BM RS; MDS\/MPN\u2010RS\u2010T patients also demonstrate <jats:italic>JAK2<\/jats:italic>V617F (50%), <jats:italic>DNMT3A<\/jats:italic>, <jats:italic>TET2<\/jats:italic> and <jats:italic>ASXL1<\/jats:italic> mutations. Cytogenetic abnormalities are uncommon in both.<\/jats:p><\/jats:sec><jats:sec><jats:title>Risk Stratification<\/jats:title><jats:p>Most patients with MDS\u2010RS\u2010SLD are stratified into lower risk groups by the revised\u2010IPSS. Disease outcome in MDS\/MPN\u2010RS\u2010T is better than that of MDS\u2010RS\u2010SLD, but worse than that of essential thrombocythemia (MPN). Both diseases are associated with a low risk of leukemic transformation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Treatment<\/jats:title><jats:p>Anemia and iron overload are complications seen in both and are managed similar to lower risk MDS and MPN. Luspatercept, a first\u2010in\u2010class erythroid maturation agent is now approved for the management of anemia in patients with MDS\u2010RS and MDS\/MPN\u2010RS\u2010T. Aspirin therapy is reasonable in MDS\/MPN\u2010RS\u2010T, especially in the presence of <jats:italic>JAK2<\/jats:italic>V617F, but the value of platelet\u2010lowering drugs remains to be defined.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ajh.26090","type":"journal-article","created":{"date-parts":[[2021,1,15]],"date-time":"2021-01-15T06:24:24Z","timestamp":1610691864000},"page":"379-394","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":55,"title":["Myelodysplastic syndromes with ring sideroblasts (<scp>MDS\u2010RS<\/scp>) and <scp>MDS<\/scp>\/myeloproliferative neoplasm with <scp>RS<\/scp> and thrombocytosis (<scp>MDS\/MPN\u2010RS\u2010T<\/scp>) \u2013 \u201c<scp>2021<\/scp> update on diagnosis, risk\u2010stratification, and management\u201d"],"prefix":"10.1002","volume":"96","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6998-662X","authenticated-orcid":false,"given":"Mrinal 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