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 |
| 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.