{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,15]],"date-time":"2026-06-15T05:42:51Z","timestamp":1781502171007,"version":"3.54.1"},"reference-count":83,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2017,2,11]],"date-time":"2017-02-11T00:00:00Z","timestamp":1486771200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2017,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Disease Overview<\/jats:title><jats:p>Ring sideroblasts (RS) are erythroid precursors with abnormal perinuclear mitochondrial iron accumulation. 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 risk MDS, with single or multilineage dysplasia (SLD\/MLD), &lt;5% bone marrow (BM) 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\u2009\u2265\u2009450 \u00d7 10(9)\/L and large atypical megakaryocytes (similar to <jats:italic>BCR\u2010ABL1<\/jats:italic> negative MPN).<\/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; with <jats:italic>ASXL1\/SETBP1<\/jats:italic> mutations adversely impacting survival. Cytogenetic abnormalities are uncommon in both diseases.<\/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\u2010International Prognostic Scoring System (R\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<\/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. 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 is uncertain.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ajh.24637","type":"journal-article","created":{"date-parts":[[2017,2,11]],"date-time":"2017-02-11T12:25:33Z","timestamp":1486815933000},"page":"297-310","source":"Crossref","is-referenced-by-count":55,"title":["Refractory anemia with ring sideroblasts (RARS) and RARS with thrombocytosis (<scp>RARS<\/scp>\u2010<scp>T<\/scp>): 2017 update on diagnosis, risk\u2010stratification, and management"],"prefix":"10.1002","volume":"92","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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