Acquired von Willebrand’s Syndrome and Acquired Thrombopathy in a Patient with a Chronic Lymphocytic Leukemia

Abstract

The patient was 65 years old when she developped spontaneous bleeding. She did not have a family history of abnormal bleeding and profered clear evidence of normal hemostasis before.Bleeding time, platelet aggregation induced by collagen were abnormal. VIII related antigen, ristocetin cofactor were 10 per cent the normal range while VIII procoagulant activity was 50%.Interaction with rabbit sub endothelium was studied: contact and adhesion were 50% of the normal range and thrombi were absent.Infusion of cryoprecipitate corrected partially von Willebrand abnormalities while platelet aggregation induced by collagen remained unchanged. Velocity of platelet aggregation induced by collagen, VIII related antigen and ristocetin cofactor increased moderately after corticotherapy.The association of an acquired thrombopathy and von Willebrand’s disease in a patient with chronic lymphocytic leukemia lead one to wonder about possible relationship between these diseases in this case.

Acquired von willebrand syndrome due to an inhibitor specific for von willebrand factor antigens

“…Acquired von Willebrand syndrome has been described in association with hematologic malignancies in four other cases. In only one was an inhibitor noted; Wautier et a1 described a patient with chronic lymphocytic leukemia with an IgA inhibitor to von Willebrand function [8]. In another patient with hairy cell leukemia, no inhibitor was detectable but an abnormal factor VIII pattern was present on crossed immunoelectrophoresis similar to our patient's findings [35].…”

Acquired von willebrand disease in patients with polycythemia rubra vera

“…Of interest is the observation that the inhibitory activity is concentrated in the IgG fraction. In most patients demonstrating the circulating inhibitors, the inhibitory activity has been found mainly in the IgG fraction [9][10][11]35,36]. The demonstration of the inhibitor with specificity for von Willebrand factor is very rare in myeloproliferative disorders [43].…”

Acquired von Willebrand disease and storage pool disease in chronic myelocytic leukemia

“…Three mechanisms to explain the pathogenesis of acquired von Willebrand disease have been proposed. The first suggests that antibodies either inactivate the biologic activities of von Willebrand factor [11][12][13]331 or induce rapid clearance of the complex from the circulation through the formation of immunocomplexes [34]. The second shows that acquired von Willebrand disease is the result of selective absorption of von Willebrand factor to malignant cells, leading to low plasma levels [14,35,36].…”