We report the case of a 60-year-old non-alcoholic male with complaints of generalized weakness, progressive pallor and abdominal discomfort over one year. Patients with this condition have clinico-morphological features of MDS (RARS) along with marked thrombocytosis and abnormal megakaryocytes similar to those seen in breakpoint cluster region-Abelson murine leukemia-1 (BCR ABL1)-negative MPN.
1 Initially named as refractory anemia with ringed sideroblasts associated with marked thrombocytosis (RARS-T), it was a provisional entity under MDS/MPN in the 2008 WHO classification of hematopoietic tumors. Myelodysplastic syndrome (MDS)-myeloproliferative neoplasm (MPN) with ringed sideroblasts and thrombocytosis (MDS/MPN-RS-T) is a rare entity categorized under the myelodysplastic-myeloproliferative group (MDS/MPN) in the recent 2016 World Health Organization (WHO) update on the classification of myeloid neoplasms.