An Acquired Von Willebrand’s Disease With IgG Monoclonal Gammopathy, Normal Bleeding Time And Positive VIIIR:AG In The Vascular Endothelium

A 71-year old female noticed nose bleeds a year ago and diagnosed to have an acquired von Willebrand’s disease with IgG, K monoclonal gammopathy. VIII:C was of 0.10 U/ml, VIIIR: AG and VIIIR: WF of both below 0.05 U/ml. Bleeding time was within normal limit by both Duke’s(5 min.) and Ivy’s (7 min.) methods. Platelet retention rate was 10% by Bowie’s method. Ristocetion induced platelet aggregation was nil at 1.2mg/ml of concentration. Serum Immunoelectrophoresis showed K type IgG M-bow, but the IgG value was within normal limit (1,407 mg/dl). Bone marrow findings were normal. Over response of VIII: C following the infusion of cryoprecipitate was not seen. Her profuse epistaxis was stopped when the cryoprecipitate was administered but not the Factor VIII concentrate. Patient plasma showed an inhibitory effect to VIII: C (1.12 Bethesda unit), VIIIR: AG and VIIIR: WF. By immunefluorescent staining VIIIR: AG was proven to be present in normal quantity in the vascular endothelium and platelet surface.