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- Title
von Willebrand factor activity levels are influenced by driver mutation status in polycythemia vera and essential thrombocythemia patients with well‐controlled platelet counts.
- Authors
Iizuka, Kazuhide; Morishita, Soji; Nishizaki, Yuji; Iizuka, Yoshikazu; Iriyama, Noriyoshi; Ochiai, Tomonori; Yanagisawa, Naotake; Yasuda, Hajime; Ando, Jun; Gotoh, Akihiko; Takei, Masami; Hatta, Yoshihiro; Nakamura, Hideki; Nakayama, Tomohiro; Komatsu, Norio
- Abstract
von Willebrand factor ristocetin cofactor (vWF activity) and platelet count (PLT) are negatively correlated in patients with polycythemia vera (PV) and essential thrombocythemia (ET). However, vWF activity does not always normalize upon controlling PLT in those patients. To address this issue, we investigated the correlation between vWF activity and PLT in PV and ET patients. The negative correlation between vWF activity and PLT was stronger in calreticulin mutation‐positive (CALR+) ET than in Janus kinase 2 mutation‐positive (JAK2+) PV or ET groups. When PLT were maintained at a certain level (<600 × 109/L), low vWF activity (<50%) was more frequently observed in JAK2+ PV patients than in JAK2+ ET (p =.013) or CALR+ ET (p =.013) groups, and in PV and ET patients with ≥50% JAK2+ allele burden than in those with allele burden <50% (p =.015). High vWF activity (>150%) was more frequent in the JAK2+ ET group than in the CALR+ ET group (p =.005), and often associated with vasomotor symptoms (p =.002). This study suggests that some patients with JAK2+ PV or ET have vWF activity outside the standard range even with well‐controlled PLT, and that the measurement of vWF activity is useful for assessing the risk of thrombosis and hemorrhage.
- Subjects
VON Willebrand factor; VON Willebrand disease; POLYCYTHEMIA vera; PLATELET count; THROMBOCYTOSIS; TRAFFIC accidents
- Publication
European Journal of Haematology, 2022, Vol 109, Issue 6, p779
- ISSN
0902-4441
- Publication type
Article
- DOI
10.1111/ejh.13866