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Clinical characteristics in adolescents and young adults with polycythemia vera and essential thrombocythemia in Japan

Sugimoto, Yuka ; Nagaharu, Keiki LU ; Ohya, Eiko ; Ohishi, Kohshi ; Tawara, Isao ; Ito, Tomoki ; Gotoh, Akihiko ; Nakamae, Mika ; Kimura, Fumihiko and Koike, Michiaki , et al. (2024) In International Journal of Hematology 120(6). p.684-693
Abstract

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... (More)

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.

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organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Adolescent and young adult (AYA), Bleeding events, Essential thrombocythemia (ET), Polycythemia vera (PV), Thrombotic events
in
International Journal of Hematology
volume
120
issue
6
pages
10 pages
publisher
Springer
external identifiers
  • scopus:85207582424
  • pmid:39467899
ISSN
0925-5710
DOI
10.1007/s12185-024-03862-5
language
English
LU publication?
yes
id
1e683efa-ca66-4da4-b07c-c628c3adcb25
date added to LUP
2024-12-18 11:31:37
date last changed
2025-07-17 04:31:59
@article{1e683efa-ca66-4da4-b07c-c628c3adcb25,
  abstract     = {{<p>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 &lt; 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 &lt; 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.</p>}},
  author       = {{Sugimoto, Yuka and Nagaharu, Keiki and Ohya, Eiko and Ohishi, Kohshi and Tawara, Isao and Ito, Tomoki and Gotoh, Akihiko and Nakamae, Mika and Kimura, Fumihiko and Koike, Michiaki and Kirito, Keita and Wada, Hideho and Usuki, Kensuke and Tanaka, Takayuki and Mori, Takehiko and Wakita, Satoshi and Saito, Toshiki I. and Saito, Akiko M. and Shimoda, Kazuya and Kurokawa, Toshiro and Tomita, Akihiro and Edahiro, Yoko and Hashimoto, Yoshinori and Kiyoi, Hitoshi and Akashi, Koichi and Matsumura, Itaru and Takenaka, Katsuto and Komatsu, Norio}},
  issn         = {{0925-5710}},
  keywords     = {{Adolescent and young adult (AYA); Bleeding events; Essential thrombocythemia (ET); Polycythemia vera (PV); Thrombotic events}},
  language     = {{eng}},
  number       = {{6}},
  pages        = {{684--693}},
  publisher    = {{Springer}},
  series       = {{International Journal of Hematology}},
  title        = {{Clinical characteristics in adolescents and young adults with polycythemia vera and essential thrombocythemia in Japan}},
  url          = {{http://dx.doi.org/10.1007/s12185-024-03862-5}},
  doi          = {{10.1007/s12185-024-03862-5}},
  volume       = {{120}},
  year         = {{2024}},
}