Department of Hematology, Erasmus University Medical Center, Rotterdam, The Netherlands.
Department of Hematology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
Br J Haematol. 2022 May;197(4):497-501. doi: 10.1111/bjh.18145. Epub 2022 Mar 22.
Von Willebrand disease (VWD) is a bleeding disorder caused by quantitative (type 1 or 3) or qualitative (type 2A/2B/2M/2N) defects of circulating von Willebrand factor (VWF). Circulating VWF levels not always fully explain bleeding phenotypes, suggesting a role for alternative factors, like platelets. Here, we investigated platelet factor 4 (PF4) in a large cohort of patients with VWD. PF4 levels were lower in type 2B and current bleeding phenotype was significantly associated with higher PF4 levels, particularly in type 1 VWD. Based on our findings we speculate that platelet degranulation and cargo release may play a role across VWD subtypes.
血管性血友病（VWD）是一种由循环性血管性血友病因子（VWF）的定量（1 型或 3 型）或定性（2A/2B/2M/2N 型）缺陷引起的出血性疾病。循环 VWF 水平并不总是能完全解释出血表型，这表明替代因素（如血小板）可能发挥作用。在这里，我们在一个大型 VWD 患者队列中研究了血小板因子 4（PF4）。2B 型患者的 PF4 水平较低，当前的出血表型与更高的 PF4 水平显著相关，尤其是在 1 型 VWD 中。基于我们的发现，我们推测血小板脱颗粒和货物释放可能在所有 VWD 亚型中都发挥作用。
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