Cannova Joseph, Shah Shiv, Patel Anand A
Section of Hematology-Oncology, Department of Medicine, University of Chicago, Chicago, IL.
Department of Medicine, University of Chicago, Chicago, IL.
Clin Lymphoma Myeloma Leuk. 2025 Jul;25(7):484-493. doi: 10.1016/j.clml.2025.02.003. Epub 2025 Feb 16.
Philadelphia-chromosome negative (Ph-neg) myeloproliferative neoplasms (MPNs) carry a variable rate of progression to the fibrotic stage of disease and/or the accelerated/blast-phase (AP/BP) of disease. Many of the challenging management scenarios that arise in the treatment of MPNs occur in those with higher-risk myelofibrosis (MF) or MPN-AP/BP. In this review we will focus upon 3 challenging clinical scenarios pertinent to the management of high-risk MPNs. We discuss how to incorporate molecular data into decision making around allogeneic hematopoietic stem cell transplantation in MF, strategies to address splenomegaly in patients with MF with inadequate response to an initial JAK inhibitor, and what sort of treatment approaches can be employed in the management of MPN-AP/BP.
费城染色体阴性（Ph阴性）骨髓增殖性肿瘤（MPN）进展为疾病纤维化阶段和/或疾病加速期/急变期（AP/BP）的发生率各不相同。MPN治疗中出现的许多具有挑战性的管理情况发生在高危骨髓纤维化（MF）或MPN-AP/BP患者中。在本综述中，我们将重点关注与高危MPN管理相关的3种具有挑战性的临床情况。我们讨论如何将分子数据纳入MF异基因造血干细胞移植的决策制定中，针对初始JAK抑制剂反应不足的MF患者脾肿大的处理策略，以及MPN-AP/BP管理中可采用何种治疗方法。
