VWF and Von Willebrand disease: AMSTAR: Assessment of Multiple Systematic Reviews; RCT: randomized clinical trial; SRL and MA: systematic review literature and meta-analysis; VWD: von Willebrand disease; DDAVP: desmopressin; PMP: platelet microparticle; PK: pharmacokinetic; PAC-1: protease-activated receptor 1; IBD: inherited bleeding disease.