| Therapy | Dose | Comments |
| Therapies directed at treatment of bleeding (or prevention of surgical bleeding) |
| DDAVP (desmopressin) | - Intravenous dose: 0.3 mcg/kg (maximum dose 20 mcg) in 50 mL saline over 20 min
| - Can be repeated every 12 to 24 hours, but tachyphylaxis occurs after 3 to 5 doses.
- Follow response at 1 and 4 hours (assay VWF and factor VIII activities).
- Follow serum sodium after 2 to 3 doses to avoid hyponatremia (may need to discontinue).
- Use with caution in patients with cardiovascular disease.
|
| VWF concentrate (plasma-derived or recombinant) | - Plasma-derived: Usual starting dose, 40 to 60 units/kg intravenously
- Recombinant: Usual starting dose, 80 units/kg intravenously
| - Follow response (VWF and factor VIII activities) at 1 and 4 hours to estimate half-life.
- Adjust dose and frequency of infusion depending on clinical course.
- Consider continuous infusion if therapy is effective but half-life is short.
|
| Antifibrinolytic agents (tranexamic acid or epsilon aminocaproic acid) | - TXA: 10 mg/kg intravenously 3 times per day or 25 mg/kg orally every 6 to 8 hours
- EACA: 25 to 50 mg/kg orally 4 times per day
| - Either agent may be used.
- Generally given as adjunctive therapy with other treatments.
- Reduce dose in patients with kidney failure.
|
| Recombinant activated factor VII (rFVIIa) | - NovoSeven RT*: Usual dose, 90 mcg/kg intravenously every 4 to 6 hours
| - Use for life-threatening bleeding after other therapies fail.
- Avoid in patients with cardiovascular risk.
|
| Therapies directed at increasing VWF levels and decreasing the levels of autoantibodies¶ |
| IVIG | - 1 gram/kg intravenously over several hours, once per day for 2 days (total dose, 2 grams/kg)
| - Most effective in IgG MGUS and in other autoantibody-mediated disease.
- May take 2 to 3 days to prevent clearance of VWF and raise VWF activity levels in patients with a response.
|
| Plasmapheresis | - One exchange every 2 to 3 days, with each exchange consisting of 1 to 1.5 plasma volumes
| - May use as a temporizing measure (with other treatments) in patients with multiple myeloma or other disorders with high paraprotein levels.
- In most cases, a total of 3 to 5 procedures.
- Use albumin as replacement fluid.
|
