Acquired Von Willebrand’s Syndrome in Systemic Lupus Erythematosus
Table 3
Therapeutic options in acquired von Willebrand syndrome associated with SLE.
Therapeutic option
Dose
Comment
Desmopressin
0.3 μg/kg over 30 minutes once or twice daily
Limited response, use in combination with IVIG
VWF containing concentrates
30–100 VWF : RCo units/kg
Dose depending on the patient’s residual activity, severity of bleeding, and presence of inhibitors; also limited response, use in combination with IVIG
Recombinant factor VIIa
90 μg/kg (range: 40–150 μg/kg) every 2–6 hours
For a median of 3 doses (range 1–54 doses)
IVIG
1 g/kg/d for 2 days
May repeat a single high-dose 1 g/kg/d every 21 days; use of desmopressin and VWF containing concentrates is recommended initially for bleeding control as response is not immediate
Steroids
Prednisone 30–40 mg/d or 1-2 mg/kg per day
It treats underlying cause
Cyclophosphamide
700 mg/m2
It treats underlying cause; single doses have been effective but treatment may be repeated monthly
Rituximab
375 mg/m2 weekly for 2–4 weeks
Has been followed by doses every 90 days as long-term management in AVWS associated with B cell lymphoma
