{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,4]],"date-time":"2026-07-04T15:51:13Z","timestamp":1783180273287,"version":"3.54.6"},"reference-count":80,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2015,5,19]],"date-time":"2015-05-19T00:00:00Z","timestamp":1431993600000},"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":[[2015,6]]},"abstract":"<jats:p><jats:bold><jats:italic>Disease Overview<\/jats:italic><\/jats:bold>: 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) and RARS with thrombocytosis (RARS\u2010T).<\/jats:p><jats:p><jats:bold><jats:italic>Diagnosis<\/jats:italic><\/jats:bold>: RARS is a lower risk myelodysplastic syndrome (MDS) with dysplasia limited to the erythroid lineage, &lt;5% bone marrow (BM) blasts and \u226515% BM RS. RARS\u2010T is a provisional entity in the MDS\/MPN (myeloproliferative neoplasm) overlap syndromes, with diagnostic features of RARS, along with a platelet count \u2265450 \u00d7 10(9)\/L and large atypical megakaryocytes similar to those observed in <jats:italic>BCR\u2010ABL1<\/jats:italic> negative MPN.<\/jats:p><jats:p><jats:bold><jats:italic>Mutations and Karyotype<\/jats:italic><\/jats:bold>: Mutations in the <jats:italic>SF3B1<\/jats:italic> gene are seen in \u226580% of patients with RARS and RARS\u2010T, and strongly correlate with the presence of BM RS; RARS\u2010T patients have additional mutations such as, <jats:italic>JAK2<\/jats:italic>V617F (\u223c60%), <jats:italic>MPL<\/jats:italic> (&lt;5%), and <jats:italic>CALR<\/jats:italic> (&lt;5%). Cytogenetic abnormalities are uncommon in both RARS and RARS\u2010T.<\/jats:p><jats:p><jats:bold><jats:italic>Risk stratification<\/jats:italic><\/jats:bold>: Most patients with RARS are stratified into lower risk groups by the International Prognostic Scoring System (IPSS) for MDS and the revised IPSS. Disease outcome in RARS\u2010T is better than that of RARS, but worse than that of essential thrombocytosis. Both RARS and RARS\u2010T have a low risk of leukemic transformation.<\/jats:p><jats:p><jats:bold><jats:italic>Treatment<\/jats:italic><\/jats:bold>: Anemia and iron overload are complications in both diseases and are managed similar to lower risk MDS. Aspirin therapy is reasonable in RARS\u2010T, especially in the presence of <jats:italic>JAK2<\/jats:italic>V617F, but the value of platelet\u2010lowering drugs is uncertain. Case reports of RARS\u2010T therapy with lenalidomide warrant additional studies. Am. J. Hematol. 90:550\u2013559, 2015. \u00a9 2015 Wiley Periodicals, Inc.<\/jats:p>","DOI":"10.1002\/ajh.24038","type":"journal-article","created":{"date-parts":[[2015,4,21]],"date-time":"2015-04-21T08:29:45Z","timestamp":1429604985000},"page":"549-559","source":"Crossref","is-referenced-by-count":39,"title":["Refractory anemia with ring sideroblasts and <scp>RARS<\/scp> with thrombocytosis"],"prefix":"10.1002","volume":"90","author":[{"given":"Mrinal M.","family":"Patnaik","sequence":"first","affiliation":[{"name":"Division of Hematology, Department of Internal Medicine Mayo Clinic Rochester Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ayalew","family":"Tefferi","sequence":"additional","affiliation":[{"name":"Division of Hematology, Department of Internal Medicine Mayo Clinic Rochester Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2015,5,19]]},"reference":[{"key":"e_1_2_6_2_1","volume-title":"WHO Classification of Tumors of Haematopoietic and Lymphoid Tissues","author":"Swederlow S","year":"2008"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1053\/j.seminhematol.2009.07.001"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2013.092395"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.C100141200"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2013-09-381665"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2002-07-2006"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.89.9.4028"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng.359"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/8.5.743"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.tig.2008.05.008"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2012-03-415356"},{"key":"e_1_2_6_13_1","volume-title":"WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues","author":"Swederlow S","year":"2008"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2000.01940.x"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0145-2126(00)00088-6"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-09-377994"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2006-02-005751"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.leu.2404405"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2009-05-222331"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1038\/leu.2014.49"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2014.119032"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1311347"},{"key":"e_1_2_6_23_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1312542"},{"key":"e_1_2_6_24_1","doi-asserted-by":"publisher","DOI":"10.1038\/leu.2014.47"},{"key":"e_1_2_6_25_1","doi-asserted-by":"publisher","DOI":"10.1038\/nature10496"},{"key":"e_1_2_6_26_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1103283"},{"key":"e_1_2_6_27_1","doi-asserted-by":"publisher","DOI":"10.1038\/leu.2011.232"},{"key":"e_1_2_6_28_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2014-03-560227"},{"key":"e_1_2_6_29_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-09-377275"},{"key":"e_1_2_6_30_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2012-05-430876"},{"key":"e_1_2_6_31_1","doi-asserted-by":"publisher","DOI":"10.1038\/leu.2012.298"},{"key":"e_1_2_6_32_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2011.053918"},{"key":"e_1_2_6_33_1","doi-asserted-by":"publisher","DOI":"10.1038\/leu.2011.320"},{"key":"e_1_2_6_34_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.23373"},{"key":"e_1_2_6_35_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1109016"},{"key":"e_1_2_6_36_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2011.40.7379"},{"key":"e_1_2_6_37_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-12-400994"},{"key":"e_1_2_6_38_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V89.6.2079"},{"key":"e_1_2_6_39_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2012-03-420489"},{"key":"e_1_2_6_40_1","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.23697"},{"key":"e_1_2_6_41_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2006.08.5696"},{"key":"e_1_2_6_42_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1013343"},{"key":"e_1_2_6_43_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2013-03-492884"},{"key":"e_1_2_6_44_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.23642"},{"key":"e_1_2_6_45_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1995.tb08909.x"},{"key":"e_1_2_6_46_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00277-008-0450-7"},{"key":"e_1_2_6_47_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2004-10-3872"},{"key":"e_1_2_6_48_1","doi-asserted-by":"publisher","DOI":"10.1634\/theoncologist.2011-S3-35"},{"key":"e_1_2_6_49_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2008.07181.x"},{"key":"e_1_2_6_50_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2007-06-096370"},{"key":"e_1_2_6_51_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa041668"},{"key":"e_1_2_6_52_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2007-01-068833"},{"key":"e_1_2_6_53_1","first-page":"586","article-title":"Sotatercept, a soluble activin receptor type 2A IgG\u2010fc fusion protein for the treatment of anemia and bone loss","volume":"12","author":"Raje N","year":"2010","journal-title":"Curr Opin Mol Ther"},{"key":"e_1_2_6_54_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-12-399667"},{"key":"e_1_2_6_55_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2362.2007.01915.x"},{"key":"e_1_2_6_56_1","doi-asserted-by":"publisher","DOI":"10.6004\/jnccn.2009.0082"},{"key":"e_1_2_6_57_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.21192"},{"key":"e_1_2_6_58_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.21391"},{"key":"e_1_2_6_59_1","doi-asserted-by":"publisher","DOI":"10.1038\/leu.2009.39"},{"key":"e_1_2_6_60_1","doi-asserted-by":"publisher","DOI":"10.1038\/leu.2010.105"},{"key":"e_1_2_6_61_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2013-04-499772"},{"key":"e_1_2_6_62_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.22001"},{"key":"e_1_2_6_63_1","doi-asserted-by":"publisher","DOI":"10.3109\/03630269.2011.578515"},{"key":"e_1_2_6_64_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.21119"},{"key":"e_1_2_6_65_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2002.04.117"},{"key":"e_1_2_6_66_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2005.05.4346"},{"key":"e_1_2_6_67_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2012.47.3314"},{"key":"e_1_2_6_68_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2004-01-0338"},{"key":"e_1_2_6_69_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2012.46.8652"},{"key":"e_1_2_6_70_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2012.072538"},{"key":"e_1_2_6_71_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2010-01-263087"},{"key":"e_1_2_6_72_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-2006-949664"},{"key":"e_1_2_6_73_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-02-339002"},{"key":"e_1_2_6_74_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2014-08-596676"},{"key":"e_1_2_6_75_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.23895"},{"key":"e_1_2_6_76_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2010-01-263319"},{"key":"e_1_2_6_77_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-06-359224"},{"key":"e_1_2_6_78_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199504273321704"},{"key":"e_1_2_6_79_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2008-03-143537"},{"key":"e_1_2_6_80_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2009.23.6075"},{"key":"e_1_2_6_81_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.leu.2404743"}],"container-title":["American Journal of Hematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fajh.24038","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajh.24038","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,23]],"date-time":"2023-09-23T14:19:20Z","timestamp":1695478760000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ajh.24038"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,5,19]]},"references-count":80,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2015,6]]}},"alternative-id":["10.1002\/ajh.24038"],"URL":"https:\/\/doi.org\/10.1002\/ajh.24038","archive":["Portico"],"relation":{},"ISSN":["0361-8609","1096-8652"],"issn-type":[{"value":"0361-8609","type":"print"},{"value":"1096-8652","type":"electronic"}],"subject":[],"published":{"date-parts":[[2015,5,19]]}}}