Acquired von Willebrand syndrome (AVWS)

Management of AVWS involves treatment of both bleeds and the underlying condition.

VWF and FVIII levels can be raised either with desmopressin or with a VWF-FVIII containing concentrate, but factor levels can be short-lived due to increased clearance.

Recombinant activated factor VII (rFVIIa) has been effective in some cases that were resistant to desmopressin or VWF-FVIII concentrates.

Administration of high dose intravenous IgG (IVIG) may prolong the half-life of VWF by interfering with clearance mechanisms. IVIG has been used in connection with treatment of bleeds and for prophylactic treatment during surgery or delivery. However, IVIG is not working if the antibody is of IgM isotype.

Plasma exchange has also been successful in some cases with a monoclonal antibody.

Extracorporeal immunoadsorption has been reported in cases with high titer inhibiting antibodies.

Immunosuppressive agents and corticosteroids are effective in some patients with autoimmune disorders or monoclonal gammopathy of undetermined significance (MGUS).

Treatment of the underlying condition may result in improved or normalized VWF levels. Complete restoration has been achieved after tumor resection, chemotherapy, radiotherapy, valve replacement, or thyroxine replacement [1,2].