Three cases of acquired von Willebrand disease associated with systemic lupus erythematosus

Acquired von Willebrand disease associated with systemic lupus erythematosu s (SLE) has been detected in three middle-aged women. In each case the firs t clinical manifestation was a bleeding syndrome. Plasma levels of von Will ebrand factor (VWF) and ristocetin-induced platelet agglutination were as f ound in type 1 von Willebrand disease for the first patient, type 3 for the second patient, and type 2 for the third patient. Intraplatelet levels of VWF were normal for all three patients. In all cases a mixture of patient's plasma with normal plasma resulted in inhibition of ristocetin-induced bin ding of VWF to normal platelets. Intravenous immunoglobulin given to patien ts 2 and 3 corrected the plasma VWF level of the second patient but not tha t of the third. Therapy with corticosteroids was partially beneficial for p atient 3 and patient 2. For patient 2, the severity of the cutaneous lesion s also led to the use of cyclophosphamide, and this therapy resulted in tot al correction of VWF levels, Our observations confirm previous reports of a cquired von Willebrand syndrome associated with SLE and show heterogeneity both in the phenotypic form and in the response to treatment.