We investigated data from 180 consecutive patients with myelodysplastic/myeloproliferative neoplasms with SF3B1 mutation and thrombocytosis (MDS/MPN-SF3B1-T) who were diagnosed according to the 2022 World Health Organization (WHO) classification of myeloid neoplasms to identify covariates associated with survival. At a median follow-up of 48 months (95% confidence interval [CI] 35–61 months), the median survival was 69 months (95% CI 59–79 months). Patients with bone marrow ring sideroblasts (RS) < 15% had shorter median overall survival (OS) than did those with bone marrow RS ≥ 15% (41 months [95% CI 32–50 months] versus 76 months [95% CI 59–93 months]; P < 0.001). According to the univariable analyses of OS, age ≥ 65 years (P < 0.001), hemoglobin concentration (Hb) < 80 g/L (P = 0.090), platelet count (PLT) ≥ 800 × 10E + 9/L (P = 0.087), bone marrow RS < 15% (P < 0.001), the Revised International Prognostic Scoring System (IPSS-R) cytogenetic category intermediate/poor/very poor (P = 0.005), SETBP1 mutation (P = 0.061) and SRSF2 mutation (P < 0.001) were associated with poor survival. Based on variables selected from univariable analyses, two separate survival prediction models, a clinical survival model, and a clinical-molecular survival model, were developed using multivariable analyses with the minimum value of the Akaike information criterion (AIC) to specifically predict outcomes in patients with MDS/MPN-SF3B1-T according to the 2022 WHO classification.

我们调查了 180 名连续患有SF3B1突变和血小板增多症的骨髓增生异常/骨髓增生性肿瘤 (MDS/MPN- SF3B1 -T) 患者的数据，这些患者根据 2022 年世界卫生组织 (WHO) 骨髓肿瘤分类进行诊断，以确定与生存相关的协变量。中位随访 48 个月（95% 置信区间 [CI] 35-61 个月），中位生存期为 69 个月（95% CI 59-79 个月）。骨髓环铁粒幼细胞 (RS) < 15% 的患者比骨髓环铁粒幼细胞 (RS) ≥ 15% 的患者中位总生存期 (OS) 更短（41 个月 [95% CI 32-50 个月]与76 个月 [95% CI 59]） –93 个月]；P  < 0.001）。根据OS单变量分析，年龄≥65岁（P  <0.001），血红蛋白浓度（Hb）<80g/L（P  =0.090），血小板计数（PLT）≥800×10E+9/L（P  = 0.087），骨髓RS < 15%（P  < 0.001），修订国际预后评分系统（IPSS-R）细胞遗传学类别中/差/极差（P  = 0.005），SETBP1突变（P  = 0.061）和SRSF2突变( P  < 0.001) 与较差的生存率相关。基于从单变量分析中选择的变量，使用多变量分析和 Akaike 信息标准 (AIC) 的最小值开发了两个独立的生存预测模型，即临床生存模型和临床分子生存模型，以专门预测患者的结果根据 2022 年 WHO 分类，具有 MDS/MPN- SF3B1 -T。