Table 1.

Summary of studies reporting continuous-infusion VWF in aVWD

Publication, n	aVWD etiology	Baseline laboratory values	Continuous-infusion indication	Continuous-infusion dose	VWF:RCo activity	Adjunctive therapies
Patient 1	IgG κ MGUS	M spike 0.33 g/dL FVIII 18% VWF Ag 10% VWF:RCo <20% VWF propeptide 92%	Epidural steroid injection	4 VWF:RCo units/kg per hour	104%	IVIG
			Lumbar radiofrequency ablation	6 VWF:RCo units/kg per hour	>150%	IVIG
Patient 2	IgG κ MGUS	M spike 0.29 g/dL FVIII 13% VWF Ag <25% VWF:RCo <20%	Gluteal hematoma	7 VWF:RCo units/kg per hour	99%	None, IVIG given 3 d later
Patient 3	IgG κ MGUS	M spike 0.7 g/dL FVIII 25% VWF Ag <5% VWF:RCo <20% VWF propeptide 149%	Ankle fracture and hemarthrosis	5 VWF:RCo units/kg per hour	135%	IVIG
Frank et al, 20025 (n = 1)	IgG MGUS	FVIII 48% VWF Ag ND VWF:RCo <20%	Total hip replacement	6 VWF:RCo units/kg per hour	35%-70%	Desmopressin, IVIG,
Lipkind et al, 20056 (n = 1)	SLE	FVIII 2% VWF:RCo <5%	Labor and delivery	7 FVIII units/kg per hour (∼3 VWF:RCo units/kg per hour)	200%	Dexamethasone, IVIG
Patel et al, 20147 (n = 1; 2 episodes)	CLL	FVIII 9% VWF Ag 16% VWF:RCo <10%	Right hip total arthroplasty and postoperative wound hematoma	15 VWF:RCo units/kg per hour	22%-76%	Antifibrinolytics
			Hand and forearm hematoma	30 VWF:RCo units/kg per hour	23%-104%	None
			Hematuria	30 VWF:RCo units/kg per hour	37%-80%	None

Ag, antigen; CLL, chronic lymphocytic leukemia; ND, not detectable; SLE, systemic lupus erythematosus.