MPL gene mutation is a possible risk factor for thrombosis in patients with essential thrombocythemia in Japan

Autor: Chiho Furuya, Yoshinori Hashimoto, Soji Morishita, Tadaaki Inano, Tomonori Ochiai, Shuichi Shirane, Yoko Edahiro, Marito Araki, Miki Ando, Norio Komatsu
Jazyk: angličtina
Rok vydání: 2023
Předmět:
Zdroj: Hematology, Vol 28, Iss 1 (2023)
Druh dokumentu: article
ISSN: 16078454
1607-8454
DOI: 10.1080/16078454.2023.2229131
Popis: ABSTRACTObjectives Since MPL mutation is a rare driver gene mutation found in a small number of essential thrombocythemia (ET) patients, the clinical characteristics of patients with MPL mutations and their association with thrombotic events have not yet been elucidated in Japan.Methods We enrolled 579 Japanese ET patients based on the diagnostic criteria of the WHO classification 2017 and compared clinical characteristics of MPL-mutated patients (n = 22; 3.8%) to JAK2V617F-mutated (n = 299; 51.6%), CALR-mutated (n = 144; 24.9%), and triple-negative (TN) (n = 114; 19.7%) patients.Results Thrombosis during follow up was observed in 4 out of 22 (18.2%) in the MPL-mutated group, which was the highest among all driver gene mutation groups (JAK2V617F-mutated, 8.7%; CALR-mutated, 3.5%; TN,1.8%). The MPL- and JAK2V617F-mutated groups had worse thrombosis-free survival (TFS) than the CALR-mutated (p = 0.043) and TN groups (p = 0.006). Univariable analysis revealed that a history of thrombosis was a possible risk factor for thrombosis among MPL-mutated patients (hazard ratio: 9.572, p = 0.032).Conclusions MPL-mutated ET patients should require more intensive management to prevent recurrence of thrombosis.
Databáze: Directory of Open Access Journals
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