| Factor VIII deficiency: recombinant factor VIII concentrate | — | Genetic deficiency | Intravenous transfusion |
| Factor IX deficiency: recombinant factor IX concentrate |
| Factor V deficiency: factor concentrate |
| Factor VII deficiency |
| Factor XIII deficiency |
| Factor XI deficiency: plasma-derived factor XI concentrate* |
| Tranexamic acid |
Lysine analogue that binds plasmin, preventing it from adhering to fibrin and thus preventing clot breakdown Adverse effects: dizziness and nausea/thrombosis may occur with other thrombotic risk factors
| Inhibits fibrinolysis |
Administered intravenously or orally, or suspended in solution for topical or mouthwash use 1000–1300 mg orally or 1000 mg intravenously 3 times per day Weight-based dosing 10 mg/kg 3 times per day
|
| Desmopressin |
Vasopressin derivative that releases stores of vWF and factor VIII from endothelial cells; can elevate plasma levels of vWF and factor VIII three- to fivefold Adverse effects: has antidiuretic hormone activity, so fluid restriction is recommended
|
Used primarily for vWD type 1, as endothelial cells must produce normal molecules Use in type 2 minor bleeding is valid
|
Administered intravenously or subcutaneously (0.3 μg/kg) for clinic or hospital-based use, or as nasal spray by outpatients (common use for heavy bleeding) A test dose to determine the size and duration of the response is recommended, as the 2 responses may vary substantially; response frequently declines with repeated use at intervals < 24–48 h
|
| vWF concentrate† | — |
vWF-containing concentrate with high-molecular-weight vWF multimers is used for treatment if desmopressin is contraindicated or if response is likely to be inadequate Has second role in hemostasis: to bind to factor VIII and protect it from premature clearance and degradation
| Intravenous transfusion |