Myelofibrotic and leukemic transformation in 2016 WHO-defined Philadelphia-negative myeloproliferative neoplasm

• Published in: Blood research Vol. 57; no. 1; pp. 59 - 68
• Main Authors: Song, Ik-Chan, Yeon, Sang Hoon, Lee, Myung-Won, Ryu, Hyewon, Lee, Hyo-Jin, Yun, Hwan-Jung, Kim, Seon Young, Jo, Deog-Yeon
• Format: Journal Article
• Language: English
• Published: Korea (South) Korean Society of Hematology; Korean Society of Blood and Marrow Transplantation; Korean Society of Pediatric Hematology-Oncology; Korean Society on Thrombosis and Hemostasis 31-03-2022; 대한혈액학회
• Subjects: Original; 병리학; Essential thrombocythemia; Polycythemia vera; Secondary myelofibrosis; Primary myelofibrosis; Leukemia; Myeloproliferative neoplasm
• ISSN: 2287-979X; 2288-0011
• 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.