Login / Signup

Treatment outcomes for patients with myelodysplastic syndrome/myeloproliferative neoplasms with ring sideroblasts and thrombocytosis.

Rami KomrokjiMegan MelodyNajla Al AliOnyee ChanVirginia KlimekBrian J BallMikkael A SekeresGeorge LucasJaroslaw P MaciejewskiDavid A SallmanEric PadronAndrew KuykendallTerra LashoAref A Al-KaliKiran NaqviDavid P SteensmaGuillermo Garcia-ManeroMirinal S Patnaik
Published in: Leukemia & lymphoma (2021)
Myelodysplastic syndrome/myeloproliferative neoplasm with ring sideroblasts and thrombocytosis (MDS/MPN-RS-T) is characterized by anemia, ring sideroblast erythroid precursors, and persistent thrombocytosis. Case reports suggest lenalidomide may be effective in treating MDS/MPN-RS-T. We evaluated a large series of patients with MDS/MPN-RS-T to compare hematological improvement (HI) response rates among different drug therapies including lenalidomide. We identified 167 patients with MDS/MPN-RS-T. Among the patients tested, 84% had SF3B1 mutations and 43% had JAK2 V617F mutations. The median OS for the cohort was 81 months. Overall, 76 patients (46%) received erythropoiesis-stimulating agents (ESAs), 47 patients (28%) received lenalidomide, and 45 patients (27%) received hypomethylating agents (HMAs). The HI rates were 58%, 53%, and 24%, respectively. The median duration of treatment was 11 months for lenalidomide compared to 6 months for HMAs. Rates of HI improvement were higher in patients with MDS/MPN-RS-T treated with ESAs or lenalidomide, in comparison to those treated with HMAs.
Keyphrases
  • newly diagnosed
  • end stage renal disease
  • chronic kidney disease
  • ejection fraction
  • stem cell transplantation
  • low dose
  • patient reported
  • chronic lymphocytic leukemia