TY - JOUR
T1 - Clinical characteristics in adolescents and young adults with polycythemia vera and essential thrombocythemia in Japan
AU - Sugimoto, Yuka
AU - Nagaharu, Keiki
AU - Ohya, Eiko
AU - Ohishi, Kohshi
AU - Tawara, Isao
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 - Saito, Akiko M.
AU - Shimoda, Kazuya
AU - Kurokawa, Toshiro
AU - Tomita, Akihiro
AU - Edahiro, Yoko
AU - Hashimoto, Yoshinori
AU - Kiyoi, Hitoshi
AU - Akashi, Koichi
AU - Matsumura, Itaru
AU - Takenaka, Katsuto
AU - Komatsu, Norio
N1 - Publisher Copyright:
© Japanese Society of Hematology 2024.
PY - 2024/12
Y1 - 2024/12
N2 - We report the first large-scale retrospective cohort study on adolescent and young adult (AYA) polycythemia vera (PV) and essential thrombocythemia (ET) in Japan, a subgroup analysis using Japanese multicenter registry data (JSH-MPN-R18). This study included patients with PV (n = 31) or ET (n = 141) aged 20 to 39 years at the initial visit. Hemorrhage-free survival (HFS) was better in AYA ET than in non-AYA ET (5-year HFS: 100% vs. 88.6%, p < 0.01), which might be attributed to differences in antithrombotic treatment rates between AYA and non-AYA patients. Although thrombosis-free survival did not differ statistically, the percentage of venous thrombotic events (TEs) among total TEs was higher in AYA compared to non-AYA PV and ET in Japan (26.0% vs. 6.0%, p < 0.01), but much lower than figures reported in European or US cohorts. Cytoreductive therapy (CRT) was administered to 25.8% of AYA patients with PV and 43.3% of AYA patients with ET, and the reason was usually unrelated to high risk of thrombosis. These results could be used to develop a more appropriate strategy for managing PV and ET in the Japanese AYA population.
AB - We report the first large-scale retrospective cohort study on adolescent and young adult (AYA) polycythemia vera (PV) and essential thrombocythemia (ET) in Japan, a subgroup analysis using Japanese multicenter registry data (JSH-MPN-R18). This study included patients with PV (n = 31) or ET (n = 141) aged 20 to 39 years at the initial visit. Hemorrhage-free survival (HFS) was better in AYA ET than in non-AYA ET (5-year HFS: 100% vs. 88.6%, p < 0.01), which might be attributed to differences in antithrombotic treatment rates between AYA and non-AYA patients. Although thrombosis-free survival did not differ statistically, the percentage of venous thrombotic events (TEs) among total TEs was higher in AYA compared to non-AYA PV and ET in Japan (26.0% vs. 6.0%, p < 0.01), but much lower than figures reported in European or US cohorts. Cytoreductive therapy (CRT) was administered to 25.8% of AYA patients with PV and 43.3% of AYA patients with ET, and the reason was usually unrelated to high risk of thrombosis. These results could be used to develop a more appropriate strategy for managing PV and ET in the Japanese AYA population.
KW - Adolescent and young adult (AYA)
KW - Bleeding events
KW - Essential thrombocythemia (ET)
KW - Polycythemia vera (PV)
KW - Thrombotic events
UR - https://www.scopus.com/pages/publications/85207582424
UR - https://www.scopus.com/pages/publications/85207582424#tab=citedBy
U2 - 10.1007/s12185-024-03862-5
DO - 10.1007/s12185-024-03862-5
M3 - Article
C2 - 39467899
AN - SCOPUS:85207582424
SN - 0925-5710
VL - 120
SP - 684
EP - 693
JO - International Journal of Hematology
JF - International Journal of Hematology
IS - 6
ER -