TY - JOUR
T1 - Real-world clinical characteristics of post-essential thrombocythemia and post-polycythemia vera myelofibrosis
AU - Shide, Kotaro
AU - Takenaka, Katsuto
AU - Kitanaka, Akira
AU - Numata, Akihiko
AU - Kameda, Takuro
AU - Yamauchi, Takuji
AU - Inagaki, Atsushi
AU - Mizuno, Shohei
AU - Takami, Akiyoshi
AU - Ito, Shinichi
AU - Hagihara, Masao
AU - Usuki, Kensuke
AU - Maekawa, Takaaki
AU - Sunami, Kazutaka
AU - Ueda, Yasunori
AU - Tsutsui, Miyuki
AU - Ando, Miki
AU - Komatsu, Norio
AU - Ozawa, Keiya
AU - Kurokawa, Mineo
AU - Arai, Shunya
AU - Mitani, Kinuko
AU - Akashi, Koichi
AU - Shimoda, Kazuya
N1 - Publisher Copyright:
© 2023, The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.
PY - 2024/1
Y1 - 2024/1
N2 - There are few prospective studies on patients with post-essential thrombocythemia myelofibrosis (PET-MF) and post-polycythemia vera myelofibrosis (PPV-MF). Therefore, we conducted a nationwide longitudinal prospective survey to clarify the clinical characteristics of these diseases. A total of 197 PET-MF and 117 PPV-MF patients diagnosed between 2012 and 2021 were analyzed. The median age at diagnosis was 70.0 years for both diseases. The time from diagnosis of ET or PV to that of MF was 9.6 and 10.4 years, respectively, with no significant difference. Patients with PPV-MF had higher hemoglobin levels and white blood cell counts than those with PET-MF, whereas those with PET-MF had higher platelet counts than those with PPV-MF. Although splenomegaly was more frequent in patients with PPV-MF at diagnosis, there was no difference in the frequency of constitutional symptoms. Ruxolitinib was the most common treatment administered to 74.6% and 83.8% of patients with PET-MF and PPV-MF, respectively. Patients with PET-MF and PPV-MF had similar prognoses, with 3-year overall survival (OS) of 0.742 in PET-MF and 0.768 in PPV-MF patients. In both diseases, leukemic transformation was the leading cause of death, followed by infection. The 3-year OS for patients with PET/PPV-MF and primary MF diagnosed during the same period was 0.754 and 0.626, respectively, with no significant difference. This survey provides real-world clinical features and prognostic data on secondary myelofibrosis in the ruxolitinib era.
AB - There are few prospective studies on patients with post-essential thrombocythemia myelofibrosis (PET-MF) and post-polycythemia vera myelofibrosis (PPV-MF). Therefore, we conducted a nationwide longitudinal prospective survey to clarify the clinical characteristics of these diseases. A total of 197 PET-MF and 117 PPV-MF patients diagnosed between 2012 and 2021 were analyzed. The median age at diagnosis was 70.0 years for both diseases. The time from diagnosis of ET or PV to that of MF was 9.6 and 10.4 years, respectively, with no significant difference. Patients with PPV-MF had higher hemoglobin levels and white blood cell counts than those with PET-MF, whereas those with PET-MF had higher platelet counts than those with PPV-MF. Although splenomegaly was more frequent in patients with PPV-MF at diagnosis, there was no difference in the frequency of constitutional symptoms. Ruxolitinib was the most common treatment administered to 74.6% and 83.8% of patients with PET-MF and PPV-MF, respectively. Patients with PET-MF and PPV-MF had similar prognoses, with 3-year overall survival (OS) of 0.742 in PET-MF and 0.768 in PPV-MF patients. In both diseases, leukemic transformation was the leading cause of death, followed by infection. The 3-year OS for patients with PET/PPV-MF and primary MF diagnosed during the same period was 0.754 and 0.626, respectively, with no significant difference. This survey provides real-world clinical features and prognostic data on secondary myelofibrosis in the ruxolitinib era.
KW - Post-essential thrombocythemia myelofibrosis
KW - Post-polycythemia vera myelofibrosis
KW - Primary myelofibrosis
KW - Ruxolitinib
UR - http://www.scopus.com/inward/record.url?scp=85175956558&partnerID=8YFLogxK
U2 - 10.1007/s00277-023-05528-4
DO - 10.1007/s00277-023-05528-4
M3 - Article
AN - SCOPUS:85175956558
SN - 0939-5555
VL - 103
SP - 97
EP - 103
JO - Annals of Hematology
JF - Annals of Hematology
IS - 1
ER -