Summary
A case with evidence of acquired von Willebrand’s syndrome associated with systemic
lupus erythmatosus and Sjögren’s syndrome is described. The patient, who had no family
history of bleeding, presented a haemorrhagic diathesis of recent origin, the bleeding
time was prolonged, procoagulant Factor-VIII and von Willebrand factor levels were
low and platelet aggregation was decreased with different concentrations of Ristocetin®.
No improvement was seen after the tranfusion of cryoprecipitates, and there was no
increase in procoagulant Factor-VIII.
Clinical improvement resulted after treatment with corticosteroids, and later, the
laboratory abnormalities characteristic of von Willebrand’s disease became normal.
The level of procoagulant factor-VIII reached the very high level of 810%.
