Predicting survival in myelodysplastic/myeloproliferative neoplasms with SF3B1 mutation and thrombocytosis

Abstract

We interrogated data from 180 consecutive subjects with myelodysplastic/myeloproliferative neoplasm with SF3B1 mutation and thrombocytosis (MDS/MPN-SF3B1-T) diagnosed according to the 2022 World Health Organization (WHO) classification of myeloid neoplasms to identify co-variates associated with survival. At a median follow-up of 45 months (Inter-Quartile Range [IQR], 19–73 months), median survival was 70 months (95% Confidence Interval [CI], 57, 84 months). Subjects with bone marrow ring sideroblasts < 15% had briefer median survival compared with those in whom it was ≥ 15% (41 months [32, 50 months] versus 80 months [63, 97 months]; P = 0.003). In multi-variable analyses, age ≥ 65 years (P = 0.005), haemoglobin concentration < 80 g/L (P = 0.008), platelet concentration ≥ 800×10E + 9/L (P = 0.02), bone marrow ring sideroblasts < 15% (P = 0.007), complex cytogenetics (P = 0.04) and SETBP1 mutation (P = 0.004) were independently associated with worse survival. Using these data we divided subjects into 3 risk cohorts: (1) low- (N = 77; median 96 months [72, 120 months]); (2) intermediate- (N = 70; median 56 months [32, 80 months]); and (3) high-risk (N = 7; median 17 months [15, 19 months]; All pairwise comparisons P < 0.0001). Our model, if validated, should help physicians estimate survival in persons with MDS/MPN-SF3B1-T in the 2022 WHO classification.