Outcomes and predictors of survival in blast phase myeloproliferative neoplasms
Outcomes and predictors of survival in blast phase myeloproliferative neoplasms
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DOI：
10.1016/j.leukres.2018.05.004
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发表时间：
2018-07-01
影响因子：
2.7
通讯作者：
Hobbs, Gabriela
中科院分区：
文献类型：
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作者：
Lancman, Guido;Brunner, Andrew;Hobbs, Gabriela
我们回顾性分析了57例急变期骨髓增生性肿瘤（MPN-BP）患者的治疗结果。整个队列的中位总生存期（OS）为5.8个月。对于接受诱导治疗的患者，67%达到完全缓解（CR），75%接受干细胞移植（SCT）。所有移植患者（n=19）在中位随访19.2个月后未达到中位OS，而非移植患者为3.8个月（p< 0.0001）;诱导化疗后未接受SCT的患者中位生存期为4.9个月。CR后移植患者的OS（中位随访26.7个月后未达到OS）与前期或初始治疗反应不佳后移植的患者相比（9.0个月; p= 0.097）没有改善。那些在MPN过程中依赖输血并接受SCT的患者的中位OS为4.4个月，所有患者均死于SCT并发症。接受低甲基化药物（HMA）的患者存活6.7个月，而接受支持治疗的患者存活1.1个月。虽然MPN-BP的结果仍然很差，但在适当选择的患者中，最好在诱导治疗获得完全缓解后，使用SCT可以实现长期生存。对于不适合SCT的患者，HMA可以提供与诱导化疗相似的生存率，毒性更低。
We retrospectively reviewed treatment outcomes for 57 patients with myeloproliferative neoplasms in blast phase (MPN-BP). The median overall survival (OS) of the entire cohort was 5.8 months. For patients receiving induction therapy, 67% achieved a complete response (CR) and 75% received stem cell transplantation (SCT). Median OS for all transplanted patients (n=19) was not reached after a median follow-up of 19.2 months compared with 3.8 months in non-transplanted patients (p< 0.0001); patients who did not receive SCT after induction chemotherapy survived a median of 4.9 months. OS was not improved in patients transplanted after CR (OS not reached after median follow-up of 26.7 months) compared with those transplanted upfront or after suboptimal response to initial therapy (9.0 months; p=.097). Those who were transfusion-dependent during their MPN course and received SCT had a median OS of 4.4 months, with all patients dying from SCT complications. Patients receiving hypomethylating agents (HMA) survived 6.7 months, while those receiving supportive care survived 1.1 months. Although outcomes for MPN-BP remain poor, long-term survival can be achieved in appropriately selected patients utilizing SCT, optimally after attaining a complete response with induction therapy. For patients ineligible for SCT, HMAs can offer similar survival to induction chemotherapy with less toxicity.