Acquired
von Willebrand disease (AvWD) is a relatively rare acquired
bleeding disorder that usually occurs in elderly patients, in whom its recognition may be delayed. Patients usually present predominantly with mucocutaneous
bleeding, with no previous history of
bleeding abnormalities and no clinically meaningful family history. Various underlying diseases have been associated with AvWD, most commonly hematoproliferative disorders, including
monoclonal gammopathies,
lymphoproliferative disorders, and
myeloproliferative disorders. The pathogenesis of AvWD remains incompletely understood but includes
autoantibodies directed against the
von Willebrand factor (vWF), leading to a more rapid clearance from the circulation or interference with its function, adsorption of vWF by
tumor cells, and nonimmunologic mechanisms of destruction. Laboratory evaluation usually reveals a pattern of prolonged bleeding time and decreased levels of vWF
antigen,
ristocetin cofactor activity, and
factor VIII coagulant activity consistent with a diagnosis of vWD. Acquired vWD is distinguished from the congenital form by age at presentation, absence of a personal and family history of
bleeding disorders, and, often, presence of a hematoproliferative or autoimmune disorder. The severity of the
bleeding varies considerably among patients. Therapeutic options include
desmopressin and certain
factor VIII concentrates that also contain vWF. Successful treatment of the associated illness can reverse the clinical and laboratory manifestations.
Intravenous immunoglobulins have also shown some efficacy in the management of AvWD, especially cases associated with
monoclonal gammopathies. Awareness of AvWD is essential for diagnosis and appropriate management.