Human Von willebrand factor(Boya Bio-Pharmaceutical)

(1) Pharmacokinetic test Main objective: To evaluate the PK characteristics of human von Willebrand factor in patients with von Willebrand disease through a single-dose pharmacokinetic (PK) test. Secondary objective: To evaluate the incremental recovery rate and activity recovery rate of human von Willebrand factor after a single dose, and to provide a basis for the calculation of clinical dosage. (2) Clinical efficacy phase trial Main objective: To evaluate the efficacy and safety of human von Willebrand factor in the treatment of spontaneous and trauma-induced bleeding in adults and adolescents (≥12 years old) with von Willebrand disease, and to provide a basis for marketing authorization application. Secondary objectives: 1) To further observe and evaluate the clinical efficacy of human von Willebrand factor in the treatment of bleeding events using other clinical secondary efficacy indicators. 2) To observe and evaluate safety during the trial, including clinical symptoms and signs and laboratory indicators, and to analyze the incidence and severity of adverse events (AEs), including serious adverse events (SAEs) and adverse reactions (ADRs). During the trial, the focus was on monitoring thrombosis-related risks and observing adverse events after each medication and local adverse reactions at the administration site.