Visweshwar Nathan, Fletcher Bradley, Jaglal Michael, Laber Damian A, Patel Ankita, Eatrides Jennifer, Rathnakumar Geetha Rajasekharan, Iyer Keshav Visweswaran, Ayala Irmel, Manoharan Arumugam
Division of Hematology, University of South Florida, Tampa, FL 33612, USA.
Department of Hematology and Hematological Oncology, Moffitt Cancer Center, Tampa, FL 33612, USA.
J Clin Med. 2024 Aug 22;13(16):4952. doi: 10.3390/jcm13164952.
Polycythemia vera is an indolent myeloproliferative disorder that predisposes patients to venous and arterial thrombosis and can transform into myelofibrosis and acute myeloid leukemia. Consistent phlebotomy prevents life-threatening cerebrovascular and coronary artery disease and prolongs survival in low-risk polycythemia vera (patients under 60 years without thrombosis). However, despite its effectiveness in preventing serious complications, phlebotomy does not necessarily enhance the quality of life (QoL). This review assesses QoL issues associated with low-risk PV, explores alternative management strategies such as erythrocytapheresis, and discusses the roles of hydroxyurea, peginterferon, ruxolitinib, and other novel agents in potentially improving disease management and patient outcomes.
真性红细胞增多症是一种进展缓慢的骨髓增殖性疾病,使患者易发生静脉和动脉血栓形成,并可转化为骨髓纤维化和急性髓系白血病。持续放血可预防危及生命的脑血管和冠状动脉疾病,并延长低危真性红细胞增多症(60岁以下无血栓形成的患者)的生存期。然而,尽管放血在预防严重并发症方面有效,但并不一定会提高生活质量(QoL)。本综述评估了与低危真性红细胞增多症相关的生活质量问题,探讨了诸如治疗性红细胞单采术等替代管理策略,并讨论了羟基脲、聚乙二醇干扰素、芦可替尼和其他新型药物在潜在改善疾病管理和患者预后方面的作用。