Haematology Department, CHU de Poitiers, Poitiers, France.
Department of Cancer Biology, Onco-Hematology Unit, CHU de Poitiers, Poitiers, France.
Br J Haematol. 2023 Jul;202(2):284-288. doi: 10.1111/bjh.18853. Epub 2023 May 14.
Myeloproliferative neoplasms in blastic phase (MPN-BP) have a dreadful prognosis. We report the characteristics and outcomes of five MPN-BP patients treated with a never-before-described combination of azacytidine and venetoclax (to control BP transformation), added to ruxolitinib (needed to control constitutional symptoms). Median age was 76 years (range 72-84), and worst performance status was 2. The overall response rate was 80%, and the complete remission rate was 40%. With median follow-up of 10.0 months (range 4.2-13.4), median overall survival was 13.4 months (95% CI 4.2-13.4). We did not detect any unexpected treatment-related toxicity, and quality of life was improved.
骨髓增生性肿瘤急变期(MPN-BP)预后极差。我们报告了 5 例 MPN-BP 患者的特征和结局,他们接受了一种从未描述过的阿扎胞苷和维奈托克联合治疗(用于控制 BP 转化),并加用了芦可替尼(用于控制全身症状)。中位年龄为 76 岁(72-84 岁),最差的体能状态为 2 分。总体缓解率为 80%,完全缓解率为 40%。中位随访时间为 10.0 个月(4.2-13.4),总生存期为 13.4 个月(95%CI 4.2-13.4)。我们未发现任何意外的治疗相关毒性,且生活质量得到改善。