Classification and treatment.†
| Type . | VWF Activity . | Ag . | RIPA . | Pattern . | Multimer Treatment . | Comments . |
|---|---|---|---|---|---|---|
| Abbreviations: DDAVP, desmopressin acetate; IV, intravenous; Nl, normal; RIPA, ristocetin-induced platelet aggregation; VWD, von Willebrand disease; VWF, von Willebrand factor. | ||||||
| †Antifibrinolytic agents such as epsilon aminocaproic acid (50 mg/kg 4 times daily for 3–5 days; maximum 20 g/d) are often used in conjunction with other therapy; they are especially useful for mucosal bleeding (e.g., in dental procedures). | ||||||
| Type 1 | ↓ | ↓ | ↓ | Uniform ↓ | DDAVP 0.3 μg/kg IV in 50 mL saline over 20 minutes, or nasal spray 300 μg for weight >50 kg or 150 μg for <50 kg Replacement VWF concentrate at 20–30 IU/kg q12h | Perform therapeutic trial first. Most type 1 respond to DDAVP. Give 1–3 doses q12h; monitor for hyponatremia. Give VWF for 3–10 days for major bleeding. Monitor lab assays and clinical status. |
| Type 2 | ||||||
| 2A | ↓↓ | ↓ | ↓ | ↓ Large and Intermediate | DDAVP as in type 1 | Many type 2A patients respond, but response may not be as marked as in type 1. Perform therapeutic trial prior to use. |
| Replacement VWF concentrate | Administer as for type 1. | |||||
| 2B | ↓↓ | ↓ | ↑ | ↓ Large | Possibly DDAVP (see comment) as in type 1 | DDAVP may worsen thrombocytopenia; perform therapeutic trial and measure platelet count. |
| Replacement VWF concentrate | Administer as for type 1 | |||||
| 2M | ↓ | ↓ | ↓ | Normal | DDAVP as in type 1 | Perform therapeutic trial |
| Replacement VWF concentrate | Administer as for type 1 | |||||
| 2N | NlNl | Nl | Normal | DDAVP as in type 1 (see comment) | T1/2 of the increased level of factor VIII may be shortened due to lack of binding by abnormal VWF. | |
| Replacement VWF concentrate | Administer as for type 1 | |||||
| Type 3 | ↓↓↓ | ↓↓↓ | ↓↓↓ | Undetectable | Replacement VWF concentrates Platelet transfusions if inadequate response to VWF replacement | Administer as for type 1; increase initial dose to 50 IU/kg |
| Type . | VWF Activity . | Ag . | RIPA . | Pattern . | Multimer Treatment . | Comments . |
|---|---|---|---|---|---|---|
| Abbreviations: DDAVP, desmopressin acetate; IV, intravenous; Nl, normal; RIPA, ristocetin-induced platelet aggregation; VWD, von Willebrand disease; VWF, von Willebrand factor. | ||||||
| †Antifibrinolytic agents such as epsilon aminocaproic acid (50 mg/kg 4 times daily for 3–5 days; maximum 20 g/d) are often used in conjunction with other therapy; they are especially useful for mucosal bleeding (e.g., in dental procedures). | ||||||
| Type 1 | ↓ | ↓ | ↓ | Uniform ↓ | DDAVP 0.3 μg/kg IV in 50 mL saline over 20 minutes, or nasal spray 300 μg for weight >50 kg or 150 μg for <50 kg Replacement VWF concentrate at 20–30 IU/kg q12h | Perform therapeutic trial first. Most type 1 respond to DDAVP. Give 1–3 doses q12h; monitor for hyponatremia. Give VWF for 3–10 days for major bleeding. Monitor lab assays and clinical status. |
| Type 2 | ||||||
| 2A | ↓↓ | ↓ | ↓ | ↓ Large and Intermediate | DDAVP as in type 1 | Many type 2A patients respond, but response may not be as marked as in type 1. Perform therapeutic trial prior to use. |
| Replacement VWF concentrate | Administer as for type 1. | |||||
| 2B | ↓↓ | ↓ | ↑ | ↓ Large | Possibly DDAVP (see comment) as in type 1 | DDAVP may worsen thrombocytopenia; perform therapeutic trial and measure platelet count. |
| Replacement VWF concentrate | Administer as for type 1 | |||||
| 2M | ↓ | ↓ | ↓ | Normal | DDAVP as in type 1 | Perform therapeutic trial |
| Replacement VWF concentrate | Administer as for type 1 | |||||
| 2N | NlNl | Nl | Normal | DDAVP as in type 1 (see comment) | T1/2 of the increased level of factor VIII may be shortened due to lack of binding by abnormal VWF. | |
| Replacement VWF concentrate | Administer as for type 1 | |||||
| Type 3 | ↓↓↓ | ↓↓↓ | ↓↓↓ | Undetectable | Replacement VWF concentrates Platelet transfusions if inadequate response to VWF replacement | Administer as for type 1; increase initial dose to 50 IU/kg |