Table 1. Acute hemostatic and endoscopic... | American Society of Hematology

Table 1.

Acute hemostatic and endoscopic interventions for GI bleeding in VWD

Intervention	Dosage	Mechanism	Cautions	Notes
Desmopressin³	0.3 µg/kg with repeat doses given as needed	Increases plasma levels of VWF and FVIII	Contraindicated in type 2B due to increased platelet binding casing thrombocytopenia; ineffective in type 3	Patients should have established response with desmopressin challenge
VWF/FVIII concentrate³	Dependant on indication, more severe bleeds have higher suggested dose.	Exogenously increases levels of VWF and FVIII	Potential for inhibitor development, infusion reactions	Suggested targets of factor VIII >50 IU/dL and VWF:RCo >50 IU/dL
TXA³	Dose varies: suggested 1-2 g IV loading dose and up to 1 g IV every 8 hours afterward until hemostasis achieved^34,36	Antifibrinolytic agent	Avoid if hematuria present
Argon plasma coagulation²⁸	N/A	Electric current conducted through argon gas	Depth of coagulation only a few millimeters
Embolization³¹	N/A	Occlusion of culprit vessel	Greater risk of complications, including ischemic bowel	Only effective if source of bleed comes from a specific blood vessel
Surgical resection²⁴	N/A	Removal of affected GI tract	Unlikely to resolve all bleeding if further angiodysplasias develop	Last resort, high complication rate
Electrocautery^8–28	N/A	Tissue and vessel destruction with direct application of electric current	Risk of perforation higher than argon plasma coagulation

Intervention	Dosage	Mechanism	Cautions	Notes
Desmopressin³	0.3 µg/kg with repeat doses given as needed	Increases plasma levels of VWF and FVIII	Contraindicated in type 2B due to increased platelet binding casing thrombocytopenia; ineffective in type 3	Patients should have established response with desmopressin challenge
VWF/FVIII concentrate³	Dependant on indication, more severe bleeds have higher suggested dose.	Exogenously increases levels of VWF and FVIII	Potential for inhibitor development, infusion reactions	Suggested targets of factor VIII >50 IU/dL and VWF:RCo >50 IU/dL
TXA³	Dose varies: suggested 1-2 g IV loading dose and up to 1 g IV every 8 hours afterward until hemostasis achieved^34,36	Antifibrinolytic agent	Avoid if hematuria present
Argon plasma coagulation²⁸	N/A	Electric current conducted through argon gas	Depth of coagulation only a few millimeters
Embolization³¹	N/A	Occlusion of culprit vessel	Greater risk of complications, including ischemic bowel	Only effective if source of bleed comes from a specific blood vessel
Surgical resection²⁴	N/A	Removal of affected GI tract	Unlikely to resolve all bleeding if further angiodysplasias develop	Last resort, high complication rate
Electrocautery^8–28	N/A	Tissue and vessel destruction with direct application of electric current	Risk of perforation higher than argon plasma coagulation

IV, intravenously; N/A, not applicable; VWF:RCo, VWF ristocetin cofactor activity.