Laboratory for Hemostasis, Inflammation & Thrombosis, Unite Mixte de Recherche 1176 INSERM, Université Paris-Saclay, Le Kremlin-Bicêtre, France.
Department of Biomedical Engineering, Case Western Reserve University, Cleveland, OH.
Blood. 2023 Jun 8;141(23):2891-2900. doi: 10.1182/blood.2022018956.
The lack of innovation in von Willebrand disease (VWD) originates from many factors including the complexity and heterogeneity of the disease but also from a lack of recognition of the impact of the bleeding symptoms experienced by patients with VWD. Recently, a few research initiatives aiming to move past replacement therapies using plasma-derived or recombinant von Willebrand factor (VWF) concentrates have started to emerge. Here, we report an original approach using synthetic platelet (SP) nanoparticles for the treatment of VWD type 2B (VWD-2B) and severe VWD (type 3 VWD). SP are liposomal nanoparticles decorated with peptides enabling them to concomitantly bind to collagen, VWF, and activated platelets. In vitro, using various microfluidic assays, we show the efficacy of SPs to improve thrombus formation in VWF-deficient condition (with human platelets) or using blood from mice with VWD-2B and deficient VWF (VWF-KO, ie, type 3 VWD). In vivo, using a tail-clip assay, SP treatment reduced blood loss by 35% in mice with VWD-2B and 68% in mice with VWF-KO. Additional studies using nanoparticles decorated with various combinations of peptides demonstrated that the collagen-binding peptide, although not sufficient by itself, was crucial for SP efficacy in VWD-2B; whereas all 3 peptides appeared necessary for mice with VWF-KO. Clot imaging by immunofluorescence and scanning electron microscopy revealed that SP treatment of mice with VWF-KO led to a strong clot, similar to those obtained in wild-type mice. Altogether, our results show that SP could represent an attractive therapeutic alternative for VWD, especially considering their long half-life and stability.
血管性血友病 (VWD) 的创新不足源于多种因素,包括疾病的复杂性和异质性,但也源于对 VWD 患者出血症状影响的认识不足。最近,一些旨在超越使用血浆衍生或重组血管性血友病因子 (VWF) 浓缩物的替代疗法的研究计划已经开始出现。在这里,我们报告了一种使用合成血小板 (SP) 纳米颗粒治疗 2B 型血管性血友病 (VWD-2B) 和严重血管性血友病 (3 型 VWD) 的原创方法。SP 是带有肽的脂质体纳米颗粒,使它们能够同时结合胶原蛋白、VWF 和激活的血小板。在体外,使用各种微流控分析,我们证明了 SP 在改善 VWF 缺乏条件下(使用人血小板)或使用 2B 型 VWD 和缺乏 VWF 的 VWD 小鼠的血液时形成血栓的功效(VWF-KO,即 3 型 VWD)。在体内,使用尾巴夹测定,SP 治疗可使 VWD-2B 小鼠的失血减少 35%,VWF-KO 小鼠的失血减少 68%。使用带有各种肽组合修饰的纳米颗粒的进一步研究表明,尽管胶原结合肽本身不足够,但对于 VWD-2B 中的 SP 功效至关重要;而对于 VWF-KO 的小鼠,所有 3 种肽似乎都是必需的。免疫荧光和扫描电子显微镜的血栓成像显示,SP 治疗 VWF-KO 小鼠导致血栓形成强烈,类似于在野生型小鼠中获得的血栓。总之,我们的结果表明 SP 可能是 VWD 的一种有吸引力的治疗替代方案,特别是考虑到它们的半衰期长且稳定。