Polycythemia vera (PV) is a relatively indolent myeloid neoplasm with median survival that exceeds 35 years in young patients, but its natural history might be interrupted by thrombotic, fibrotic, or leukemic events, with respective 20-year rates of 26%, 16%, and 4%. Current treatment strategies in PV have not been shown to prolong survival or lessen the risk of leukemic or fibrotic progression and instead are directed at preventing thrombotic complications. In the latter regard, two risk categories are considered: high (age >60 years or thrombosis history) and low (absence of both risk factors). All patients require phlebotomy to keep hematocrit below 45% and once-daily low-dose aspirin, in the absence of contraindications. Cytoreductive therapy is recommended for high-risk or symptomatic low-risk disease; our first-line drug of choice in this regard is hydroxyurea but we consider pegylated interferon as an alternative in certain situations, including in young women of reproductive age, in patients manifesting intolerance or resistance to hydroxyurea therapy, and in situations where treatment is indicated for curbing phlebotomy requirement rather than preventing thrombosis. Additional treatment options include busulfan and ruxolitinib; the former is preferred in older patients and the latter in the presence of symptoms reminiscent of post-PV myelofibrosis or protracted pruritus. Our drug choices reflect our appreciation for long-term track record of safety, evidence for reduction of thrombosis risk, and broader suppression of myeloproliferation. Controlled studies are needed to clarify the added value of twice- vs once-daily aspirin dosing and direct oral anticoagulants. In this invited review, we discuss our current approach to diagnosis, prognostication, and treatment of PV in general, as well as during specific situations, including pregnancy and splanchnic vein thrombosis.

真性红细胞增多症 (PV) 是一种相对惰性的骨髓肿瘤，年轻患者的中位生存期超过 35 年，但其自然病程可能会被血栓、纤维化或白血病事件中断，20 年发生率分别为 26%、16%和 4%。目前的真性红斑狼疮治疗策略尚未被证明可以延长生存期或降低白血病或纤维化进展的风险，而是针对预防血栓并发症。对于后者，考虑两个风险类别：高（年龄 >60 岁或血栓形成史）和低（不存在两种风险因素）。在没有禁忌症的情况下，所有患者都需要进行静脉切开术以将血细胞比容保持在 45% 以下，并每日一次服用小剂量阿司匹林。对于高风险或有症状的低风险疾病，建议进行细胞减灭治疗；在这方面，我们选择的一线药物是羟基脲，但我们认为聚乙二醇干扰素在某些情况下可以作为替代药物，包括育龄年轻女性、对羟基脲治疗表现出不耐受或耐药的患者，以及需要治疗的情况限制放血需求而不是预防血栓形成。其他治疗选择包括白消安和鲁索替尼；前者适用于老年患者，后者适用于出现类似PV后骨髓纤维化或长期瘙痒症状的患者。我们的药物选择反映了我们对长期安全记录、降低血栓形成风险的证据以及更广泛地抑制骨髓增殖的重视。需要对照研究来阐明两次与每日一次阿司匹林给药和直接口服抗凝剂的附加值。 在这篇特邀综述中，我们讨论了目前 PV 的诊断、预测和治疗方法，以及在特定情况下（包括妊娠和内静脉血栓形成）的方法。