Authors: | Komrokji, R.; Melody, M.; al Ali, N.; Chan, O.; Klimek, V.; Ball, B. J.; Sekeres, M. A.; Lucas, G.; Maciejewski, J. P.; Sallman, D. A.; Padron, E.; Kuykendall, A.; Lasho, T.; Al-Kali, A.; Naqvi, K.; Steensma, D. P.; Garcia-Manero, G.; Patnaik, M. M. |
Article Title: | Treatment outcomes for patients with myelodysplastic syndrome/myeloproliferative neoplasms with ring sideroblasts and thrombocytosis |
Abstract: | 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. © 2021 Informa UK Limited, trading as Taylor & Francis Group. |
Keywords: | adult; cancer survival; controlled study; treatment outcome; treatment response; aged; gene mutation; human cell; major clinical study; overall survival; somatic mutation; antianemic agent; janus kinase 2; lenalidomide; drug efficacy; treatment duration; erythroid precursor cell; hemoglobin; retrospective study; wild type; myelodysplastic syndrome; azacitidine; myeloproliferative neoplasm; thrombocytosis; high throughput sequencing; decitabine; human; male; female; article; mixed myelodysplastic myeloproliferative disease; hematological improvement; mds/mpn-rs-t |
Journal Title: | Leukemia and Lymphoma |
Volume: | 63 |
Issue: | 1 |
ISSN: | 1042-8194 |
Publisher: | Taylor & Francis Group |
Date Published: | 2022-01-01 |
Start Page: | 199 |
End Page: | 204 |
Language: | English |
DOI: | 10.1080/10428194.2021.1971217 |
PUBMED: | 34448437 |
PROVIDER: | scopus |
DOI/URL: | |
Notes: | Article -- Export Date: 1 February 2022 -- Source: Scopus |