New England Journal of Medicine · 2020 · 537 citations · 29 references
MedicineHematologyPharmacotherapyImmunoregulationImmunotherapyTransfusion MedicineMyelodysplastic SyndromesPlatelet TransfusionRed Blood Cell TransfusionSideroblastic AnemiaTransfusion SupportErythropoiesis-stimulating Agent UseErythropoiesis-stimulating TherapyLower-risk Myelodysplastic SyndromesMyelodysplastic Syndrome TherapyMyelodysplastic Syndrome TreatmentRecombinant Fusion ProteinsRevised International Prognostic Scoring SystemTransfusion Dependence
BACKGROUND: Patients with anemia and lower-risk myelodysplastic syndromes in whom erythropoiesis-stimulating agent therapy is not effective generally become dependent on red-cell transfusions. Luspatercept, a recombinant fusion protein that binds transforming growth factor β superfamily ligands to reduce SMAD2 and SMAD3 signaling, showed promising results in a phase 2 study. METHODS: In a double-blind, placebo-controlled, phase 3 trial, we randomly assigned patients with very-low-risk, low-risk, or intermediate-risk myelodysplastic syndromes (defined according to the Revised International Prognostic Scoring System) with ring sideroblasts who had been receiving regular red-cell transfusions to receive either luspatercept (at a dose of 1.0 up to 1.75 mg per kilogram of body weight) or placebo, administered subcutaneously every 3 weeks. The primary end point was transfusion independence for 8 weeks or longer during weeks 1 through 24, and the key secondary end point was transfusion independence for 12 weeks or longer, assessed during both weeks 1 through 24 and weeks 1 through 48. RESULTS: Of the 229 patients enrolled, 153 were randomly assigned to receive luspatercept and 76 to receive placebo; the baseline characteristics of the patients were balanced. Transfusion independence for 8 weeks or longer was observed in 38% of the patients in the luspatercept group, as compared with 13% of those in the placebo group (P<0.001). A higher percentage of patients in the luspatercept group than in the placebo group met the key secondary end point (28% vs. 8% for weeks 1 through 24, and 33% vs. 12% for weeks 1 through 48; P<0.001 for both comparisons). The most common luspatercept-associated adverse events (of any grade) included fatigue, diarrhea, asthenia, nausea, and dizziness. The incidence of adverse events decreased over time. CONCLUSIONS: Luspatercept reduced the severity of anemia in patients with lower-risk myelodysplastic syndromes with ring sideroblasts who had been receiving regular red-cell transfusions and who had disease that was refractory to or unlikely to respond to erythropoiesis-stimulating agents or who had discontinued such agents owing to an adverse event. (Funded by Celgene and Acceleron Pharma; MEDALIST ClinicalTrials.gov number, NCT02631070; EudraCT number, 2015-003454-41.).
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International Scoring System for Evaluating Prognosis in Myelodysplastic Syndromes
Peter L. Greenberg, Christopher Cox, Michelle M. LeBeau et al. · Blood · 1997 · 4.5K citations · Full text
Revised International Prognostic Scoring System for Myelodysplastic Syndromes
Peter L. Greenberg, Heinz Tuechler, Julie Schanz et al. · Blood · 2012 · 3.1K citations · Full text
Interpreting the significance of changes in health-related quality-of-life scores.
David Osoba, George Rodrigues, James D. Myles et al. · Journal of Clinical Oncology · 1998 · 2.9K citations