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

被引:1
作者
Shide, Kotaro [1 ]
Takenaka, Katsuto [2 ]
Kitanaka, Akira [3 ]
Numata, Akihiko [4 ]
Kameda, Takuro [1 ]
Yamauchi, Takuji [4 ]
Inagaki, Atsushi [5 ]
Mizuno, Shohei [6 ]
Takami, Akiyoshi [6 ]
Ito, Shinichi [7 ]
Hagihara, Masao [8 ]
Usuki, Kensuke [9 ]
Maekawa, Takaaki [10 ]
Sunami, Kazutaka [11 ]
Ueda, Yasunori [12 ]
Tsutsui, Miyuki [13 ]
Ando, Miki [13 ]
Komatsu, Norio [13 ]
Ozawa, Keiya [14 ]
Kurokawa, Mineo [15 ]
Arai, Shunya [15 ]
Mitani, Kinuko [16 ]
Akashi, Koichi [4 ]
Shimoda, Kazuya [1 ]
机构
[1] Univ Miyazaki, Fac Med, Dept Internal Med, Div Hematol Diabet & Endocrinol, 5200 Kihara Kiyotake, Miyazaki 8891692, Japan
[2] Ehime Univ, Grad Sch Med, Dept Hematol Clin Immunol & Infect Dis, Toon, Ehime, Japan
[3] Kawasaki Med Sch, Dept Lab Med, Kurashiki, Japan
[4] Kyushu Univ, Grad Sch Med Sci, Dept Med & Biosyst Sci, Fukuoka, Japan
[5] Nagoya City West Med Ctr, Dept Hematol & Oncol, Nagoya, Japan
[6] Aichi Med Univ, Sch Med, Dept Internal Med, Div Hematol, Nagakute, Japan
[7] Hakodate City Hosp, Dept Hematol, Hakodate, Japan
[8] Eiju Gen Hosp, Dept Hematol, Tokyo, Japan
[9] NTT Med Ctr Tokyo, Dept Hematol, Tokyo, Japan
[10] Natl Def Med Coll, Dept Internal Med, Div Hematol Med, Tokorozawa, Japan
[11] Natl Hosp Org, Okayama Med Ctr, Dept Hematol, Okayama, Japan
[12] Kurashiki Cent Hosp, Dept Hematol Oncol, Kurashiki, Okayama, Japan
[13] Juntendo Univ, Sch Med, Dept Hematol, Tokyo, Japan
[14] Jichi Med Univ, Dept Med, Div Hematol, Shimotsuke, Tochigi, Japan
[15] Univ Tokyo, Grad Sch Med, Dept Hematol & Oncol, Tokyo, Japan
[16] Dokkyo Med Univ, Dept Hematol & Oncol, Mibu, Tochigi, Japan
关键词
Post-essential thrombocythemia myelofibrosis; Post-polycythemia vera myelofibrosis; Primary myelofibrosis; Ruxolitinib; CYTOGENETIC-RISK CATEGORIZATION; SURVIVAL; CONSENSUS;
D O I
10.1007/s00277-023-05528-4
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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.
引用
收藏
页码:97 / 103
页数:7
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