Arya Yajur, Syal Arshi, Gupta Monica, Gaba Saurabh
Internal Medicine, Government Medical College and Hospital, Chandigarh, Chandigarh, IND.
Cureus. 2021 Mar 30;13(3):e14193. doi: 10.7759/cureus.14193.
Treatment modalities for polycythemia vera (PV) have evolved over time. Phlebotomy and low-dose aspirin suffice in low-risk patients, but cytoreductive therapies are indicated in all high-risk patients (age ≥ 65 years or those with a history of PV-related thrombotic event) and may be considered for low-risk patients with progressively increasing splenomegaly, progressively increasing leucocyte and platelet counts, and for those who do not tolerate phlebotomy. Hydroxyurea/hydroxycarbamide or interferons can be used as first-line drugs. Hydroxyurea may not be tolerated by some patients, and it also carries risk of myelosuppression. Interferon alfa is especially useful for PV symptoms, and the newer preparation, ropeginterferon alfa-2b, has lesser incidence of flu-like reactions. Ruxolitinib reduces the JAK2V617F mutation burden and is used as a second-line drug. Anagrelide reduces platelet production and can be used in conjunction with hydroxyurea in patients with excessive thrombocytosis. The alkylating agent, busulfan, can also be used as a last resort in patients with a limited life expectancy. Prospective future treatments include givinostat, a histone deacetylase inhibitor, and idasanutlin, a murine double minute 2 antagonist.
真性红细胞增多症（PV）的治疗方式随时间不断演变。对于低风险患者，放血疗法和低剂量阿司匹林就足够了，但所有高风险患者（年龄≥65岁或有PV相关血栓形成事件病史者）均需进行细胞减灭疗法，对于脾肿大逐渐加重、白细胞和血小板计数逐渐升高的低风险患者，以及无法耐受放血疗法的患者，也可考虑使用细胞减灭疗法。羟基脲/羟基脲或干扰素可作为一线药物使用。一些患者可能无法耐受羟基脲，而且它还存在骨髓抑制风险。α干扰素对PV症状特别有效，新型制剂聚乙二醇化干扰素α-2b的流感样反应发生率较低。芦可替尼可降低JAK2V617F突变负荷，用作二线药物。阿那格雷可减少血小板生成，可与羟基脲联合用于血小板增多症患者。烷化剂白消安也可作为预期寿命有限患者的最后手段。未来的前瞻性治疗包括组蛋白去乙酰化酶抑制剂givinostat和鼠双微体2拮抗剂idasanutlin。
