Evidence-based recommendations for treating AVWS
| Underlying disorder . | Pathophysiology . | Causal treatment . | Additional treatment options . |
|---|---|---|---|
| Autoantibodies | Antibody-mediated increased clearance of VWF or inhibition of VWF function. | Steroids, cyclophosphamide, immunosuppressive therapy. | DDAVP, VWF concentrates, IVIg (2c). |
| Malignancy | Adsorbtion of VWF to tumor cells, particularly high-molecular-weight multimers. | Appropriate treatment of underlying cancer (resection, chemotherapy, radiation). | DDAVP, VWF concentrates (2c). |
| Hypothyroidism | Decreased production of VWF. | Thyroid hormone replacement. | DDAVP, VWF concentrates (2c). |
| Cardiovascular disease | Increased shear stress leading to activation of VWF and exposure of cleavage sites and reduction of high-molecular-weight multimers. | Repair of underlying heart lesion. | DDAVP, VWF concentrates (2c). |
| Valproic acid | Decreased production of VWF | Discontinuation of valproic acid | VWF concentrates (2c) |
| Underlying disorder . | Pathophysiology . | Causal treatment . | Additional treatment options . |
|---|---|---|---|
| Autoantibodies | Antibody-mediated increased clearance of VWF or inhibition of VWF function. | Steroids, cyclophosphamide, immunosuppressive therapy. | DDAVP, VWF concentrates, IVIg (2c). |
| Malignancy | Adsorbtion of VWF to tumor cells, particularly high-molecular-weight multimers. | Appropriate treatment of underlying cancer (resection, chemotherapy, radiation). | DDAVP, VWF concentrates (2c). |
| Hypothyroidism | Decreased production of VWF. | Thyroid hormone replacement. | DDAVP, VWF concentrates (2c). |
| Cardiovascular disease | Increased shear stress leading to activation of VWF and exposure of cleavage sites and reduction of high-molecular-weight multimers. | Repair of underlying heart lesion. | DDAVP, VWF concentrates (2c). |
| Valproic acid | Decreased production of VWF | Discontinuation of valproic acid | VWF concentrates (2c) |
1c, strong recommendation, low- or very-low-quality evidence; 2c, weak recommendation, low- or very-low-quality evidence.