Clinical findings:
(1) adult onset of a bleeding disorder
(2) negative family history of bleeding diathesis
(3) negative personal history for a similar bleeding diathesis
(4) presence of a condition associated with acquired disease
Conditions associated with acquired von Willebrand's disease:
(1) monoclonal gammopathy (multiple myeloma, Waldenstrom's macroglobulinemia, MGUS)
(2) lymphoproliferative disorder (non-Hodgkin's lymphoma, CLL, hairy cell leukemia)
(3) myeloproliferative disorders (polycythemia rubra vera, primary thrombocythemia)
(4) other malignancies (Wilm's tumor, adrenal carcinoma, adenocarcinomas)
(5) autoimmune disease
(6) drug-related (ciprofloxacin, griseofulvin, valproic acid, hydroxyethyl starch)
(7) uncertain
Laboratory findings:
(1) bleeding time: markedly prolonged
(2) aPTT: prolonged; mixing studies may show correction (antibody complexes not cleared in vitro)
(3) ristocetin cofactor activity: low or absent
(4) von Willebrand factor antigen: normal to low
(5) electropheresis: most often there is a selective loss of high molecular weight multimers
(6) a circulating antibody to von Willebrand factor antigen or a monoclonal protein may be present
Therapy:
(1) Stop any implicated drugs.
(2) Treat underlying condition.
(3) Intravenous immunoglobulin may help in patients with lymphoproliferative disorder or monoclonal gammopathy.
