Certain diseases may be associated with a substance (antibody, inhibitor or other compound) that binds or interferes with the function of von Willebrand's factor. This results in the clinical picture similar to that of inherited von Willebrand's disease.


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



(1) Stop any implicated drugs.

(2) Treat underlying condition.

(3) Intravenous immunoglobulin may help in patients with lymphoproliferative disorder or monoclonal gammopathy.


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