Table 6.

Therapeutic agents for von Willebrand disease (vWD).

AgentComments
Desmopressin (DDAVP) Useful in most cases of type 1 disease, occasionally in type 2 (may worsen thrombocytopenia in type 2B) 
Antifibrinolytic agents For mucosal or cutaneous bleeding; tranexamic acid is better tolerated orally than aminocaproic acid 
Combination oral contraceptives Used only for menorrhagia 
vWF/VIII concentrates Now commonly labeled and prescribed in von Willebrand factor (vWF) (ristocetin cofactor) units 
High purity vWF concentrate In clinical trials 
Cryoprecipitate Recommended only if lyophilized concentrates are not available 
Fibrin sealants or topical thrombin Often useful in surface bleeding and dental extraction; risk of antibodies to thrombin and/or factor V 
Platelet concentrates Specific replacement of platelet vWF in severe deficiency; rarely necessary 
Recombinant factor VIIa Theoretically useful; has been used in acquired vWD 
AgentComments
Desmopressin (DDAVP) Useful in most cases of type 1 disease, occasionally in type 2 (may worsen thrombocytopenia in type 2B) 
Antifibrinolytic agents For mucosal or cutaneous bleeding; tranexamic acid is better tolerated orally than aminocaproic acid 
Combination oral contraceptives Used only for menorrhagia 
vWF/VIII concentrates Now commonly labeled and prescribed in von Willebrand factor (vWF) (ristocetin cofactor) units 
High purity vWF concentrate In clinical trials 
Cryoprecipitate Recommended only if lyophilized concentrates are not available 
Fibrin sealants or topical thrombin Often useful in surface bleeding and dental extraction; risk of antibodies to thrombin and/or factor V 
Platelet concentrates Specific replacement of platelet vWF in severe deficiency; rarely necessary 
Recombinant factor VIIa Theoretically useful; has been used in acquired vWD 
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