关于加速期和爆发期骨髓增生性肿瘤的最新进展：我们正在取得进展吗？
Updates on accelerated and blast phase myeloproliferative neoplasms: Are we making progress?
发表日期：2023 Aug 01
作者：
Dina Mahdi, Jessica Spiers, Alexandros Rampotas, Nicola Polverelli, Donal P McLornan
来源：
BRITISH JOURNAL OF HAEMATOLOGY
摘要：
加速和爆发期骨髓增生性肿瘤的管理方法对医生和患者来说都是具有挑战性的。尽管有许多治疗进展，但对于不能进行异基因造血干细胞移植的患者来说，他们的预后通常非常差。这些爆发期患者的预计存活率常常报告为不到6个月。目前尚不存在能够准确预测风险和转化时间的特定免疫、基因组或临床病理学特征，这常常在患者和医生中引起高度焦虑。在本综述文章中，我们提供了关于加速和爆发期疾病潜在发病机制的最新认识的总结，并讨论当前的治疗方法和现实预后。最后，我们讨论引入更多新型药物进入临床领域后的前景。© 2023 British Society for Haematology and John Wiley & Sons Ltd.
Management approaches for accelerated and blast phase myeloproliferative neoplasms remain challenging for clinicians and patients alike. Despite many therapeutic advances, outcomes for those patients who are not allogeneic haematopoietic cell transplant eligible remain, in general, very poor. Estimated survival rates for such blast phase patients is frequently reported as less than 6 months. No specific immunological, genomic or clinicopathological signature currently exists that accurately predicts the risk and timing of transformation, which frequently induces a high degree of anxiety among patients and clinicians alike. Within this review article, we provide an up-to-date summary of current understanding of the underlying pathogenesis of accelerated and blast phase disease and discuss current therapeutic approaches and realistic outcomes. Finally, we discuss how the horizon may look with the introduction of more novel agents into the clinical arena.© 2023 British Society for Haematology and John Wiley & Sons Ltd.