von Willebrand factor activity levels are influenced by driver mutation status in polycythemia vera and essential thrombocythemia patients with well-controlled platelet counts
von Willebrand factor activity levels are influenced by driver mutation status in polycythemia vera and essential thrombocythemia patients with well-controlled platelet counts
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血小板计数控制良好的真性红细胞增多症和原发性血小板增多症患者的冯维勒布兰德因子活性水平受驱动突变状态的影响
DOI：
10.1111/ejh.13866
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发表时间：
2022
影响因子：
3.1
通讯作者：
Norio Komatsu
中科院分区：
文献类型：
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作者：
Kazuhide Iizuka;Soji Morishita;Yuji Nishizaki;Yoshikazu Iizuka;Noriyoshi Iriyama;Tomonori Ochiai;Naotake Yanagisawa;Hajime Yasuda;Jun Ando;Akihiko Gotoh;Masami Takei;Yoshihiro Hatta;Hideki Nakamura;Tomohiro Nakayama;Norio Komatsu
在真性红细胞增多症（PV）和原发性血小板增多症（ET）患者中，血管性血友病因子（vWF）活性与血小板计数（PLT）呈负相关。然而，在这些患者中，vWF活性并不总是在控制PLT后正常化。为了解决这个问题，我们研究了PV和ET患者vWF活性与PLT之间的相关性。钙网蛋白突变阳性（CALR+）ET组vWF活性与PLT的负相关性强于Janus激酶2突变阳性（JAK 2+）PV组或ET组。当PLT维持在一定水平（<600 × 109/L）时，JAK 2 + PV患者vWF活性降低（<50%）的发生率高于JAK 2 + ET（p= 0.013）或CALR + ET（p= 0.013）组，JAK 2+等位基因负荷≥ 50%的PV和ET患者vWF活性降低的发生率高于等位基因负荷<50%的患者（p= 0.015）。高vWF活性（>150%）在JAK 2 + ET组比CALR + ET组更常见（p= 0.005），并且经常与血管扩张症状相关（p= 0.002）。这项研究表明，即使PLT控制良好，一些JAK 2 + PV或ET患者的vWF活性也超出标准范围，并且vWF活性的测量有助于评估血栓形成和出血的风险。
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 incalreticulinmutation‐positive (CALR+) ET than inJanus kinase 2mutation‐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 inJAK2+ PV patients than inJAK2+ ET (p= .013) orCALR+ 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 theJAK2+ ET group than in theCALR+ ET group (p= .005), and often associated with vasomotor symptoms (p= .002). This study suggests that some patients withJAK2+ 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.