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KMID : 1142020220570010059
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Blood Research
2022 Volume.57 No. 1 p.59 ~ p.68
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Myelofibrotic and leukemic transformation in 2016 WHO-defined Philadelphia-negative myeloproliferative neoplasm
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Song Ik-Chan
Yeon Sang-Hoon
Lee Myung-Won
Ryu Hye-Won
Lee Hyo-Jin
Yun Hwan-Jung
Kim Seon-Young
Jo Deog-Yeon
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Abstract
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Background: Information on myelofibrotic and leukemic transformations in Korean Philadelphia chromosome- negative myeloproliferative neoplasms (Ph? MPNs) is limited.
Methods: 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.
Results: 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; P =0.031) and PV (12.2%; P =0.003). The 10-year incidence of leukemic transformation was significantly higher in PMF than in ET (40.0% and 7.9%, respectively; P =0.046), pre-PMF (4.7%; P =0.048), and PV (3.2%; P =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; P =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; P=0.027) but did not differ from that of patients with PMF (57%; P=0.744).
Conclusion: The rates and clinical courses of myelofibrotic and leukemic transformations in Korean patients with Ph? MPN did not differ from those in Western populations.
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KEYWORD
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Myeloproliferative neoplasm, Essential thrombocythemia, Polycythemia vera, Primary myelofibrosis, Secondary myelofibrosis, Leukemia
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