Table 4.

Antifibrinolytic agents for the treatment of von Willebrand disease

Formulation	Available concentration	Dosea
Tranexamic acid intravenous	10 mg/ml	0.5–1 g, 2–3x daily (1 ml/min) Children ≥1 year: 20 mg/kg/day in 2–3 doses a day
Tranexamic acid oral	650 mg (US) 500 mg (EU)	0.5–1 g in 2–4 doses a day Children ≥1 year: 20 mg/kg/day, in 2–3 doses a day
Tranexamic acid mouth rinse	50 mg/ml	0.5–1.5 g (15–25 mg/kg), in 2–3 doses a day “swish and swallow or spit” Children ≥1 year: 20 mg/kg/day, in 2–3 doses a day
Aminocaproic acid intravenous	250 mg/ml	Starting dose: 4–5 g slowly during the first hour, followed by continuous infusion of 1 g/h Children: 100 mg/kg or 3 g/m2 slowly (>1 h), followed by continuous infusion of 33.3 mg/kg/h or 1 g/m2/h
Aminocaproic acid oral	500 mg and 1000 mg	Starting dose: 4–5 g, followed by 1–1.25 g/h or 4–6 g every 4–6 h, with a max. dose of 24 g/day Children: starting 100 mg/kg, followed by 3 g/m2 during the first hour, followed by 33.3 mg/kg or 1 g/m2 every hour. Max. dose: 18 g/m2/day or 600 mg/kg/day
Aminocaproic acid mouth rinse	250 mg/ml	Starting dose: 4–5 g, followed by 1–1.25 g/h, with a max. dose of 24 g/day “swish and swallow or spit” Children: starting 100 mg/kg, followed by 3 g/m2 during the first hour, followed by 33.3 mg/kg or 1 g/m2 every hour. Max. dose: 18 g/m2/day or 600 mg/kg/day

^aData derived from [84]