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Two myeloid neoplasms defined by the presence of RS, include refractory anemia with ring sideroblasts (RARS), now classified under myelodysplastic syndromes with RS (MDS\u2010RS) and RARS with thrombocytosis (RARS\u2010T); now called myelodysplastic\/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\u2010risk MDS, with single or multilineage dysplasia (SLD\/MLD), &lt;5% bone marrow (BM) blasts and\u2009\u2265\u200915% 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 10(9)\/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, <jats:italic>ASXL1, DNMT3A<\/jats:italic>, <jats:italic>SETBP1<\/jats:italic>, and <jats:italic>TET2<\/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\u2010risk 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. Both diseases have 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\u2010risk MDS and MPN. The advent of luspatercept, a first\u2010in\u2010class erythroid maturation agent will tremendously boost the ability to manage anemia. 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 uncertain.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ajh.25397","type":"journal-article","created":{"date-parts":[[2019,1,9]],"date-time":"2019-01-09T03:48:21Z","timestamp":1547005701000},"page":"475-488","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":40,"title":["Refractory anemia with ring sideroblasts (RARS) and RARS with thrombocytosis: \u201c2019 Update on Diagnosis, Risk\u2010stratification, and Management\u201d"],"prefix":"10.1002","volume":"94","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6998-662X","authenticated-orcid":false,"given":"Mrinal M.","family":"Patnaik","sequence":"first","affiliation":[{"name":"Division of Hematology, Department of Internal Medicine Mayo Clinic  Rochester 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