TY - JOUR
T1 - Treatment outcomes for patients with myelodysplastic syndrome/myeloproliferative neoplasms with ring sideroblasts and thrombocytosis
AU - Komrokji, Rami
AU - Melody, Megan
AU - al Ali, Najla
AU - Chan, Onyee
AU - Klimek, Virginia
AU - Ball, Brian J.
AU - Sekeres, Mikkael A.
AU - Lucas, George
AU - Maciejewski, Jaroslaw P.
AU - Sallman, David A.
AU - Padron, Eric
AU - Kuykendall, Andrew
AU - Lasho, Terra
AU - Al-Kali, Aref
AU - Naqvi, Kiran
AU - Steensma, David P.
AU - Garcia-Manero, Guillermo
AU - Patnaik, Mrinal M.
N1 - Publisher Copyright:
© 2021 Informa UK Limited, trading as Taylor & Francis Group.
PY - 2022
Y1 - 2022
N2 - 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.
AB - 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.
KW - MDS/MPN-RS-T
KW - hematological improvement
KW - lenalidomide
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U2 - 10.1080/10428194.2021.1971217
DO - 10.1080/10428194.2021.1971217
M3 - Article
C2 - 34448437
AN - SCOPUS:85113699152
SN - 1042-8194
VL - 63
SP - 199
EP - 204
JO - Leukemia and Lymphoma
JF - Leukemia and Lymphoma
IS - 1
ER -