Table 2. Comparison of VWD treatment... | American Society of Hematology

Table 2.

Comparison of VWD treatment options

	Route of administration	Advantages	Disadvantages	Typical dosing
Desmopressin	Intranasal, IV, SQ	Easily given at home	Fluid restriction required	0.3 mcg/kg IV or 2 sprays IN (>50 kg)/1 spray IN (<50 kg)
Desmopressin	Intranasal, IV, SQ	Easily given at home	Not effective for all VWD types	0.3 mcg/kg IV or 2 sprays IN (>50 kg)/1 spray IN (<50 kg)
Plasma-derived VWF concentrates	IV	Most products contains both VWF and FVIII	Plasma product	50-60 ristocetin cofactor activity units/kg for major surgery, depending on baseline VWF level and desired goal level
Plasma-derived VWF concentrates	IV	Most products contains both VWF and FVIII	Most products contain both VWF and FVIII
Recombinant VWF concentrate	IV	Recombinant, allows titration of FVIII level	May require addition of recombinant FVIII for emergency treatment	50-80 ristocetin cofactor activity units/kg for major surgery, depending on baseline VWF level and desired goal level; for emergency treatment may require addition of recombinant FVIII depending on patient’s endogenous FVIII level
Antifibrinolytics	PO, IV	Easily given at home	May not work for nonmucosal bleeds	Aminocaproic acid: loading dose of 100 mg/kg then 50 mg/kg every 6 h
Antifibrinolytics	PO, IV	Easily given at home	May not work for nonmucosal bleeds	Tranexemic acid: 1500 mg 3 times daily × 5 d for menorrhagia

	Route of administration	Advantages	Disadvantages	Typical dosing
Desmopressin	Intranasal, IV, SQ	Easily given at home	Fluid restriction required	0.3 mcg/kg IV or 2 sprays IN (>50 kg)/1 spray IN (<50 kg)
Desmopressin	Intranasal, IV, SQ	Easily given at home	Not effective for all VWD types	0.3 mcg/kg IV or 2 sprays IN (>50 kg)/1 spray IN (<50 kg)
Plasma-derived VWF concentrates	IV	Most products contains both VWF and FVIII	Plasma product	50-60 ristocetin cofactor activity units/kg for major surgery, depending on baseline VWF level and desired goal level
Plasma-derived VWF concentrates	IV	Most products contains both VWF and FVIII	Most products contain both VWF and FVIII
Recombinant VWF concentrate	IV	Recombinant, allows titration of FVIII level	May require addition of recombinant FVIII for emergency treatment	50-80 ristocetin cofactor activity units/kg for major surgery, depending on baseline VWF level and desired goal level; for emergency treatment may require addition of recombinant FVIII depending on patient’s endogenous FVIII level
Antifibrinolytics	PO, IV	Easily given at home	May not work for nonmucosal bleeds	Aminocaproic acid: loading dose of 100 mg/kg then 50 mg/kg every 6 h
Antifibrinolytics	PO, IV	Easily given at home	May not work for nonmucosal bleeds	Tranexemic acid: 1500 mg 3 times daily × 5 d for menorrhagia

IN, intranasal; IV, intravenous; PO, oral