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 - 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.
KW - JAK2
KW - Japan
KW - Nationwide study
KW - Polycythemia vera
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U2 - 10.1007/s12185-022-03412-x
DO - 10.1007/s12185-022-03412-x
M3 - Article
C2 - 35809214
AN - SCOPUS:85133729385
SN - 0925-5710
VL - 116
SP - 696
EP - 711
JO - International Journal of Hematology
JF - International Journal of Hematology
IS - 5
ER -