Treatment

What are the two pillars of treatment for a patent with AVWS?

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Treat bleeding if present
Treat the underlying disease

AVWS, acquired von Willebrand syndrome

For management of bleeding in a patient with acquired von Willebrand syndrome (AVWS), what are reasonable options?

DDAVP (desmopressin)

DDAVP has been reported to be effective in patients with AVWS, and is considered part of first-line therapy. However, the response is not sustained.

Anti-fibrinolytics

Used as adjunctive therapy with desmopressin or vWF/FVIII concentrates. Can be administered orally, intravenously, or topically. Contraindicated in case of genitourinary bleeding.

von Willebrand factor/FVIII concentrates

May be used as first-line therapy. Response rates vary according to the underlying disorder.

Cryoprecipitate

Aspirin

Other management options for active bleeding include:

Intravenous immunoglobulin (IVIG)
Recombinant activated factor VII (FVIIa)

Why is the response to DDAVP and VWF concentrates often short lasting?

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Because many causes (e.g., autoimmune and cardiac) of AVWS are associated with increased clearance of vWF

In this case, the patient’s postoperative bleeding had resolved and he did not require vWF/FVIII concentrates or DDAVP. He was treated for his Waldenstrom’s macroglobulinemia (WM) with the hope that this would improve the acquired von Willebrand syndrome (AVWS). The patient did not have any symptoms of hyperviscosity from his WM and he was not actively bleeding, so he did not require plasmapheresis. Instead, he received combination therapy with dexamethasone, rituximab and cyclophosphamide. Over the next 6 months, his IgM levels decreased and his vWF antigen and activity levels increased. He did not have recurrence of iron deficiency anemia.

Consider a different patient with acquired von Willebrand syndrome (AVWS) resulting from essential thrombocythemia. His platelet count is 1,600 x 10⁹/L. He is being treated for this essential thrombocythemia with hydroxyurea and low dose aspirin per clinical practice guideline recommendations.

Should the patient continue taking aspirin?

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No, it is generally recommended to hold aspirin when the platelet count is over 1,500 x 10^9/L in patients with myeloproliferative disorders

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