A paradigm shift in the treatment of patients with polycythemia vera. The initial early use of recombinant interferon-alpha.

In some patients, 2-5 years of disease-modifying treatment resulted in symptom relief, regression of splenomegaly, normalization of abnormal blood counts and marrow morphology, and sustained JAK2V617F molecular remission.

在一些患者中，2-5年的疾病修饰性治疗带来了症状缓解，脾肿大回退，异常血细胞计数和骨髓形态正常化，以及持续的JAK2V617F分子缓解。

The PROUD-CONTI study showed the superiority of rIFNα compared to hydroxyurea (HU), which led to the European Medicines Agency approval in 2019 of ropeginterferon alpha-2b ("ropegIFN") for ELN-defined high-risk PV patients.

PROUD-CONTI 研究显示了 rIFNα 相比于羟基脲 (HU) 的优越性，这导致了2019年欧洲药品管理局批准了 ropeginterferon alpha-2b ("ropegIFN") 用于 ELN 定义的高危真性红细胞增多症 (PV) 患者。

In 2021, the Food and Drug Administration (USA) gave unrestricted approval, except for pregnant PV patients.

2021年，美国食品药品监督管理局 (FDA) 除了对怀孕的 PV 患者外，给予了无限制批准。

The Low-PV randomized trial established the superiority of ropegIFN compared to phlebotomy-only (PHLEB-O) in ELN-defined low-risk patients.

Low-PV 随机试验确立了 ropegIFN 相比仅采用静脉切开术 (PHLEB-O) 在 ELN 定义的低危患者中的优越性。

Nevertheless, worldwide, HU remains the cytoreductive drug most often used; maintenance PHLEB-O is still endorsed as maintenance therapy by some hematologists.

然而，在全球范围内，HU 仍然是最常使用的细胞减少药物；一些血液科医生仍然推荐维持静脉切开术 (PHLEB-O) 作为维持治疗。

The National Comprehensive Cancer Network (NCCN, USA) approved ropegIFN as a II-A recommendation for ELN-defined low and high-risk disease; the ELN suggested criteria for using rIFNα in low-risk patients if certain issues developed after PHLEB-O, including more symptoms, progressive splenomegaly, significant leukocytosis, thrombocytosis, or inadequate hematocrit control.

美国国家综合癌症网络（NCCN）批准了ropegIFN作为ELN定义的低危和高危疾病的II-A级推荐；ELN建议在PHLEB-O后出现某些问题的低危患者使用rIFNα，包括更多症状、进行性脾大、显著白细胞增多、血小板增多或造血控制不良。

These issues are important because even low-risk patients are at increased thrombotic risk, estimated at 2 to 3 times that of the general population.

这些问题很重要，因为即使是低危患者，其血栓形成风险也比普通人群高出2到3倍。

Moreover, as PV progresses, the development of myelofibrosis is the leading cause of morbidity, perhaps abetted by PHLEB-O.

此外，随着真性红细胞增多症（PV）的进展，骨髓纤维化的发展是主要的致病原因，可能由静脉曲张（PHLEB-O）加剧。

Here, we review recent progress in the treatment of PV with rIFNα and discuss our rationales and perspectives for, and the endorsement of the initial treatment with rIFNα of both low and high-risk PV patients, unless a contraindication exists to its use.

在这里，我们回顾了使用重组干扰素α（rIFNα）治疗PV的最新进展，并讨论了我们对低危和高危PV患者初始使用rIFNα治疗的理由和观点，除非存在对其使用的禁忌症。