Real-world clinical characteristics of post-essential thrombocythemia and post-polycythemia vera myelofibrosis.

Autor: Shide K; Division of Hematology, Diabetes, and Endocrinology, Department of Internal Medicine, Faculty of Medicine, University of Miyazaki, 5200 Kihara Kiyotake, Miyazaki, 889-1692, Japan., Takenaka K; Department of Hematology, Clinical Immunology, and Infectious Diseases, Ehime University Graduate School of Medicine, Toon, Ehime, Japan., Kitanaka A; Department of Laboratory Medicine, Kawasaki Medical School, Kurashiki, Japan., Numata A; Department of Medicine and Biosystemic Science, Kyushu University Graduate School of Medical Science, Fukuoka, Japan., Kameda T; Division of Hematology, Diabetes, and Endocrinology, Department of Internal Medicine, Faculty of Medicine, University of Miyazaki, 5200 Kihara Kiyotake, Miyazaki, 889-1692, Japan., Yamauchi T; Department of Medicine and Biosystemic Science, Kyushu University Graduate School of Medical Science, Fukuoka, Japan., Inagaki A; Department of Hematology and Oncology, Nagoya City West Medical Center, Nagoya, Japan., Mizuno S; Division of Hematology, Department of Internal Medicine, Aichi Medical University School of Medicine, Nagakute, Japan., Takami A; Division of Hematology, Department of Internal Medicine, Aichi Medical University School of Medicine, Nagakute, Japan., Ito S; Department of Hematology, Hakodate City Hospital, Hakodate, Japan., Hagihara M; Department of Hematology, Eiju General Hospital, Tokyo, Japan., Usuki K; Department of Hematology, NTT Medical Center Tokyo, Tokyo, Japan., Maekawa T; Division of Hematology, Department of Internal Medicine, National Defense Medical College, Tokorozawa, Japan., Sunami K; Department of Hematology, National Hospital Organization Okayama Medical Center, Okayama, Japan., Ueda Y; Department of Hematology/Oncology, Kurashiki Central Hospital, Okayama, Japan., Tsutsui M; Department of Hematology, Juntendo University School of Medicine, Tokyo, Japan., Ando M; Department of Hematology, Juntendo University School of Medicine, Tokyo, Japan., Komatsu N; Department of Hematology, Juntendo University School of Medicine, Tokyo, Japan., Ozawa K; Division of Hematology, Department of Medicine, Jichi Medical University, Shimotsuke, Tochigi, Japan., Kurokawa M; Department of Hematology and Oncology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan., Arai S; Department of Hematology and Oncology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan., Mitani K; Department of Hematology and Oncology, Dokkyo Medical University, Mibu, Tochigi, Japan., Akashi K; Department of Medicine and Biosystemic Science, Kyushu University Graduate School of Medical Science, Fukuoka, Japan., Shimoda K; Division of Hematology, Diabetes, and Endocrinology, Department of Internal Medicine, Faculty of Medicine, University of Miyazaki, 5200 Kihara Kiyotake, Miyazaki, 889-1692, Japan. kshimoda@med.miyazaki-u.ac.jp.
Jazyk: angličtina
Zdroj: Annals of hematology [Ann Hematol] 2024 Jan; Vol. 103 (1), pp. 97-103. Date of Electronic Publication: 2023 Nov 10.
DOI: 10.1007/s00277-023-05528-4
Abstrakt: 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.
(© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)
Databáze: MEDLINE
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