Abstract
The presence of a JAK2 V617F or JAK2 exon 12 mutation is one of the three major criteria listed for the diagnosis of polycythemia vera (PV) in the 2017 World Health Organization Classification. However, a nationwide study has not yet been conducted in Japan since the discovery of JAK2 mutations. Therefore, the Japanese Society of Hematology (JSH) retrospectively analyzed the clinical characteristics of 596 Japanese patients with PV diagnosed between April 2005 and March 2018. Among the 473 patients with complete data on JAK2 mutations available, 446 (94.3%) and 10 (2.1%) were positive for the JAK2 V617F and JAK2 exon 12 mutations, respectively. During a median follow-up of 46 months (range: 0–179 months), 47 (7.9%) deaths occurred. The major causes of death were secondary malignancies (23.4%), acute leukemia (12.8%), non-leukemic progressive disease (10.6%) and thrombotic (6.4%) and hemorrhagic complications (6.4%). Thrombotic and hemorrhagic events occurred during the clinical course in 4.0% (n = 24) and 3.5% (n = 21) of patients, respectively. These results show that the international PV prognostic score (age, venous thrombosis and leukocytosis) is applicable to Japanese patients with PV.
| Original language | English |
|---|---|
| Pages (from-to) | 696-711 |
| Number of pages | 16 |
| Journal | International Journal of Hematology |
| Volume | 116 |
| Issue number | 5 |
| DOIs | |
| Publication status | Published - 11-2022 |
All Science Journal Classification (ASJC) codes
- Hematology
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Clinical characteristics of Japanese patients with polycythemia vera : results of the JSH-MPN-R18 study. / Edahiro, Yoko; Ito, Tomoki; Gotoh, Akihiko et al.
In: International Journal of Hematology, Vol. 116, No. 5, 11.2022, p. 696-711.Research output: Contribution to journal › Article › peer-review
TY - JOUR
T1 - Clinical characteristics of Japanese patients with polycythemia vera
T2 - results of the JSH-MPN-R18 study
AU - Edahiro, Yoko
AU - Ito, Tomoki
AU - Gotoh, Akihiko
AU - Nakamae, Mika
AU - Kimura, Fumihiko
AU - Koike, Michiaki
AU - Kirito, Keita
AU - Wada, Hideho
AU - Usuki, Kensuke
AU - Tanaka, Takayuki
AU - Mori, Takehiko
AU - Wakita, Satoshi
AU - Saito, Toshiki I.
AU - Kada, Akiko
AU - Saito, Akiko M.
AU - Shimoda, Kazuya
AU - Sugimoto, Yuka
AU - Kurokawa, Toshiro
AU - Tomita, Akihiro
AU - Hashimoto, Yoshinori
AU - Akashi, Koichi
AU - Matsumura, Itaru
AU - Takenaka, Katsuto
AU - Komatsu, Norio
N1 - Funding Information: Yoko Edahiro reports research funds under contract from Meiji Seika Pharma, and an endowed chair funded by PharmaEssentia Japan K.K. Tomoki Ito reports honoraria from Novartis Pharmaceutical, Sanofi, and Takeda Pharmaceutical, and honoraria and grants from Abbvie Inc. and Bristol-Meyer Squib. Akihiko Gotoh reports honoraria from Alexion Pharma and Novartis Pharmaceutical, and grants from Chugai Pharmaceutical, Eisai Pharmaceutical, Nippon Shinyaku Pharmaceutical, Ono Pharmaceutical, Taiho Pharmaceutical, and Takeda Pharmaceutical. Mika Nakamae reports research fund from Veritas Corporation, honoraria from Novartis Pharmaceutical, and honoraria to her spouse from Novartis Pharmaceutical, Takeda Pharmaceutical, Bristol-Meyer Squib, Amgen Astellas BioPharma, Astellas Pharma, Otsuka Pharmaceutical, Celgene, Daiichi Sankyo, Nippon Shinyaku Pharmaceutical, Pfizer, Sumitomo Pharma, and Ono Pharmaceutical. Fumihiko Kimura reports grants from Ono Pharmaceutical and Takeda Pharmaceutical. Keita Kirito reports honoraria from Novartis Pharmaceutical. Hideho Wada reports grants from Chugai Pharmaceutical, Kyowa Kirin, Ono Pharmaceutical, and Takeda Pharmaceutical. Kensuke Usuki reports research funds under contract from Abbvie Inc., Amgen Astellas BioPharma, Apellis Pharmaceutical, Astellas Pharma, Bristol-Meyer Squib, Daiichi Sankyo, Janssen Pharmaceutical, Nippon Shinyaku Pharmaceutical, Otsuka Pharmaceutical, SymBio Pharmaceutical, and Takeda Pharmaceutical, and honoraria and research funds under contract from Novartis Pharmaceutical. Takehiko Mori reports honoraria from Pfizer, and research funds under contract from Asahi Kasei Pharma, Bristol-Meyer Squib, Eisai Pharmaceutical, Chugai Pharmaceutical, Kyowa Kirin, and Otsuka Pharmaceutical. Kazuya Shimoda reports honoraria from Bristol-Meyer Squib, Celgene, Novartis Pharmaceutical, and Takeda Pharmaceutical, research funds under contract from PharmaEssentia Japan K.K., and grants from Abbvie Inc., Astellas Pharma, Chugai Pharmaceutical, Kyowa Kirin, and MSD. Yuka Sugimoto reports honoraria from Novartis Pharmaceutical, research funds under contract from Takara Bio Inc., grants from Astellas Pharma, Kyowa Kirin, and Ono Pharmaceutical, and an endowed chair funded by Shojunkai Takeuchi Hospital. Akihiro Tomita reports honoraria from Takeda Pharmaceutical, research funds under contract from Kyowa Kirin, Novartis Pharmaceutical, Ono Pharmaceutical, Perseus Proteomics Inc., Pfizer, and Taiho Pharmaceutical, and honoraria and research funds under contract from Chugai Pharmaceutical. Yoshinori Hashimoto reports honoraria from Takeda Pharmaceutical, research funds under contract from Meiji Seika Pharma, and an endowed chair funded by PharmaEssentia Japan K.K. Koichi Akashi reports honoraria from Celgene and Novartis Pharmaceutical, grants from Asahi Kasei Pharma, Mochida, MSD, Mundi Pharma, Nippon Shinyaku Pharmaceutical, Ono Pharmaceutical, Shionogi, Sanofi, Sumitomo Dainippon Pharma, Taiho Pharmaceutical, Toyama Chemical, Shin Nippon Biomedical Laboratories, Yakult Honsha, and Takeda Pharmaceutical, honoraria and research funds under contract from Bristol-Meyer Squib and Janssen Pharmaceutical, honoraria and grants from Abbvie Inc., Chugai Pharmaceutical, and Eisai Pharmaceutical, research funds under contract and grants from Daiichi Sankyo and Otsuka Pharmaceutical, honoraria, research funds under contract, and grants from Astellas Pharma and Kyowa Kirin. Itaru Matsumura reports honoraria from Amgen Astellas BioPharma, Bristol-Meyer Squib, Daiichi Sankyo, Ono Pharmaceutical, SymBio Pharmaceuticals, AstraZeneca, and Janssen Pharmaceutical, research funds under contract from Alexion Pharma, grants from Kyowa Kirin, Mitsubishi Tanabe Pharma, MSD, Nippon Shinyaku Pharmaceutical, Ono Pharmaceutical, Sanofi, Shionogi, Sumitomo Dainippon Pharma, and Taiho Pharmaceutical, honoraria and research funds under contract from Pfizer, honoraria and grants from Astellas Pharma and Otsuka Pharmaceutical, research funds under contract and grants from Chugai Pharmaceutical, Eisai Pharmaceutical, and Takeda Pharmaceutical, honoraria, research funds under contract, and grants from Abbvie Inc. and Novartis Pharmaceutical. Katsuto Takenaka reports honoraria from Novartis Pharmaceutical and MSD, grants from Astellas Pharma, Chugai Pharmaceutical, and Otsuka Pharmaceutical, and honoraria and grants from Kyowa Kirin. Norio Komatsu reports honoraria from Abbvie Inc., Celgene, and Japan Tobacco Inc., research funds under contract from FUJIFILM Wako Chemicals, Fuso-pharm, Meiji Seika Pharma, Perseus Proteomics Inc., and Pfizer, grants from Bristol-Meyer Squib, Chugai Pharmaceutical, Kyowa Kirin, and Sumitomo Dainippon Pharma, honoraria and grants from Novartis Pharmaceutical and Otsuka Pharmaceutical, honoraria, research funds under contract, and grants from Takeda Pharmaceutical, honoraria, research funds under contract, and an endowed chair funded by PharmaEssentia Japan K.K. The other authors declare that they have no conflicts of interest to declare. Funding Information: We sincerely thank all the institutions that participated into this study; First Department of Internal Medicine, Kansai Medical University; Department of Hematology, Tokyo Medical University; Hematology, Graduate School of Medicine, Osaka City University; Division of Hematology, Department of Internal Medicine, National Defense Medical College; Department of Hematology, Juntendo University Shizuoka Hospital; Department of Hematology & Oncology, University of Yamanashi; Department of Hematology, Kawasaki Medical School; Department of Hematology, NTT Medical Center Tokyo; Department of Hematology, Tottori Prefectural Central Hospital; Department of Hematology, Clinical Immunology and Infectious Diseases, Ehime University Graduate School of Medicine; Division of Hematology, Department of Medicine, Keio University School of Medicine; Division of Hematology, Department of Internal Medicine, Nippon Medical School; Department of Hematology, Juntendo University Nerima Hospital; Department of Hematology, Showa University Hospital; Division of Hematology, Diabetes, and Endocrinology, Department of Internal Medicine, University of Miyazaki; Department of Hematology, Sapporo Hokuyu Hospital; Department of Hematology, Chiba University Hospital; Department of Hematology, Juntendo University Urayasu Hospital; Department of Hematology, Hyogo College of Medicine Hospital; Department of Hematology, Osaka City General Hospital; Department of Community Hematology, Mie University; Department of Hematology, Aomori Prefectural Central Hospital; Department of Hematology, Saitama Medical University International Medical Center; Department of Hematology, National Hospital Organization Kyushu Cancer Center; Department of Hematology, Gunma University Hospital; Department of Hematology, Kyoto University Hospital; Department of Hematology, Osaka Saiseikai Nakatsu Hospital; Department of Hematology, PL Hospital; Department of Hematology, Toyama Red Cross Hospital; Department of Hematology, Aiiku Hospital; Department of Third Internal Medicine, Yamagata University Hospital; Department of Hematology, Iwate Prefectural Central Hospital; Department of Hematology, Niigata University Medical & Dental Hospital; Department of Hematology and Oncology, Dokkyo University Medical Hospital; Nagoya City West Medical Center Hematology and Oncology; Department of Hematology, Saitama Medical Center; Department of Hematology, Nagasaki University Hospital; Department of Hematology, Chiba Aoba Municipal Hospital; Department of Hematology, Takeuchi Hospital; Department of Hematology, Otaru General Hospital; Department of Hematology, Juntendo University School of Medicine. Publisher Copyright: © 2022, Japanese Society of Hematology.
PY - 2022/11
Y1 - 2022/11
N2 - The presence of a JAK2 V617F or JAK2 exon 12 mutation is one of the three major criteria listed for the diagnosis of polycythemia vera (PV) in the 2017 World Health Organization Classification. However, a nationwide study has not yet been conducted in Japan since the discovery of JAK2 mutations. Therefore, the Japanese Society of Hematology (JSH) retrospectively analyzed the clinical characteristics of 596 Japanese patients with PV diagnosed between April 2005 and March 2018. Among the 473 patients with complete data on JAK2 mutations available, 446 (94.3%) and 10 (2.1%) were positive for the JAK2 V617F and JAK2 exon 12 mutations, respectively. During a median follow-up of 46 months (range: 0–179 months), 47 (7.9%) deaths occurred. The major causes of death were secondary malignancies (23.4%), acute leukemia (12.8%), non-leukemic progressive disease (10.6%) and thrombotic (6.4%) and hemorrhagic complications (6.4%). Thrombotic and hemorrhagic events occurred during the clinical course in 4.0% (n = 24) and 3.5% (n = 21) of patients, respectively. These results show that the international PV prognostic score (age, venous thrombosis and leukocytosis) is applicable to Japanese patients with PV.
AB - The presence of a JAK2 V617F or JAK2 exon 12 mutation is one of the three major criteria listed for the diagnosis of polycythemia vera (PV) in the 2017 World Health Organization Classification. However, a nationwide study has not yet been conducted in Japan since the discovery of JAK2 mutations. Therefore, the Japanese Society of Hematology (JSH) retrospectively analyzed the clinical characteristics of 596 Japanese patients with PV diagnosed between April 2005 and March 2018. Among the 473 patients with complete data on JAK2 mutations available, 446 (94.3%) and 10 (2.1%) were positive for the JAK2 V617F and JAK2 exon 12 mutations, respectively. During a median follow-up of 46 months (range: 0–179 months), 47 (7.9%) deaths occurred. The major causes of death were secondary malignancies (23.4%), acute leukemia (12.8%), non-leukemic progressive disease (10.6%) and thrombotic (6.4%) and hemorrhagic complications (6.4%). Thrombotic and hemorrhagic events occurred during the clinical course in 4.0% (n = 24) and 3.5% (n = 21) of patients, respectively. These results show that the international PV prognostic score (age, venous thrombosis and leukocytosis) is applicable to Japanese patients with PV.
UR - http://www.scopus.com/inward/record.url?scp=85133729385&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85133729385&partnerID=8YFLogxK
U2 - 10.1007/s12185-022-03412-x
DO - 10.1007/s12185-022-03412-x
M3 - Article
C2 - 35809214
AN - SCOPUS:85133729385
VL - 116
SP - 696
EP - 711
JO - International Journal of Hematology
JF - International Journal of Hematology
SN - 0925-5710
IS - 5
ER -