Song Ik-Chan, Yeon Sang Hoon, Lee Myung-Won, Ryu Hyewon, Lee Hyo-Jin, Yun Hwan-Jung, Kim Seon Young, Jo Deog-Yeon
Division of Hematology/Oncology, Department of Internal Medicine, Daejeon, Korea.
Department of Laboratory Medicine, Chungnam National University College of Medicine, Daejeon, Korea.
Blood Res. 2022 Mar 31;57(1):59-68. doi: 10.5045/br.2021.2021209.
Information on myelofibrotic and leukemic transformations in Korean Philadelphia chromosome- negative myeloproliferative neoplasms (Ph MPNs) is limited.
This study retrospectively analyzed transformations in patients diagnosed with essential thrombocythemia (ET), polycythemia vera (PV) prefibrotic/early primary myelofibrosis (pre-PMF), or overt primary myelofibrosis (PMF) based on the 2016 World Health Organization criteria between January 1996 and December 2020 at Chungam National University Hospital, Daejeon, Korea.
A total of 351 patients (144 with ET, 131 with PV, 45 with pre-PMF, and 31 with PMF; 204 men and 147 women) with a median age of 64 years (range, 15‒91 years) were followed for a median of 4.6 years (range, 0.2‒24.8 years). The 10-year incidence of overt myelofibrosis was higher in pre-PMF than in ET (31.3% and 13.7%, respectively; =0.031) and PV (12.2%; =0.003). The 10-year incidence of leukemic transformation was significantly higher in PMF than in ET (40.0% and 7.9%, respectively; =0.046), pre-PMF (4.7%; =0.048), and PV (3.2%; =0.031). The 5-year incidence of leukemic transformation was higher in patients with secondary myelofibrosis (SMF) than in those with PMF (19.0% and 11.4%, respectively; =0.040). The 5-year overall survival of patients with SMF was significantly worse than that of patients with pre-PMF (74% and 93%, respectively; =0.027) but did not differ from that of patients with PMF (57%; =0.744).
The rates and clinical courses of myelofibrotic and leukemic transformations in Korean patients with Ph MPN did not differ from those in Western populations.
关于韩国费城染色体阴性骨髓增殖性肿瘤（Ph MPNs）中骨髓纤维化和白血病转化的信息有限。
本研究回顾性分析了1996年1月至2020年12月期间在韩国大田忠南国立大学医院根据2016年世界卫生组织标准诊断为原发性血小板增多症（ET）、真性红细胞增多症（PV）纤维化前期/早期原发性骨髓纤维化（pre-PMF）或明显原发性骨髓纤维化（PMF）的患者的转化情况。
共纳入351例患者（ET患者144例、PV患者131例、pre-PMF患者45例、PMF患者31例；男性204例，女性147例），中位年龄64岁（范围15 - 91岁），中位随访时间4.6年（范围0.2 - 24.8年）。明显骨髓纤维化的10年发生率在pre-PMF中高于ET（分别为31.3%和13.7%；P = 0.031）及PV（12.2%；P = 0.003）。白血病转化的10年发生率在PMF中显著高于ET（分别为40.0%和7.9%；P = 0.046）、pre-PMF（4.7%；P = 0.048）及PV（3.2%；P = 0.031）。继发性骨髓纤维化（SMF）患者白血病转化的5年发生率高于PMF患者（分别为19.0%和11.4%；P = 0.040）。SMF患者的5年总生存率显著低于pre-PMF患者（分别为74%和93%；P = 0.027），但与PMF患者（57%；P = 0.744）无差异。
韩国Ph MPN患者骨髓纤维化和白血病转化的发生率及临床病程与西方人群无异。
