Division of Hematology & Medical Oncology, Department of Internal Medicine, Soonchunhyang University Seoul Hospital, Seoul, Korea.
Division of Hematology, Department of Internal Medicine, Konkuk University Medical Center, Seoul, Korea.
Korean J Intern Med. 2023 Nov;38(6):810-817. doi: 10.3904/kjim.2023.333. Epub 2023 Nov 1.
Myeloproliferative neoplasms (MPNs) are clonal disorders of hematopoietic stem cells. The malignant clones produce cytokines that drive self-perpetuating inflammatory responses and tend to transform into more aggressive clones, leading to disease progression. The progression of MPNs follows a biological sequence from the early phases of malignancy, polycythemia vera, and essential thrombocythemia, to advanced myelofibrosis and leukemic transformation. To date, the treatment of MPNs has focused on preventing thrombosis by decreasing blood cell counts and relieving disease-related symptoms. However, interferon (IFN) has been used to treat MPNs because of its ability to attack cancer cells directly and modulate the immune system. IFN also has the potential to modulate diseases by inhibiting JAK2 mutations, and recent studies have demonstrated clinical and molecular improvements. Long-acting IFN is administered less frequently and has fewer adverse effects than conventional IFN. The current state of research on long-acting IFN in patients with MPNs is discussed, along with future directions.
骨髓增殖性肿瘤（MPNs）是造血干细胞的克隆性疾病。恶性克隆产生细胞因子，驱动自我持续的炎症反应，并倾向于转化为更具侵袭性的克隆，导致疾病进展。MPNs 的进展遵循一个生物学序列，从恶性肿瘤的早期阶段，真性红细胞增多症和原发性血小板增多症，到晚期骨髓纤维化和白血病转化。迄今为止，MPNs 的治疗主要集中在通过降低血细胞计数和缓解与疾病相关的症状来预防血栓形成。然而，干扰素（IFN）已被用于治疗 MPNs，因为它具有直接攻击癌细胞和调节免疫系统的能力。IFN 还具有通过抑制 JAK2 突变来调节疾病的潜力，最近的研究表明了临床和分子上的改善。长效 IFN 的给药频率较低，不良反应较少。本文讨论了 MPNs 患者长效 IFN 的研究现状及未来方向。
