ACQUIRED VON WILLEBRANDS-SYNDROME IN ASSOCIATION WITH A LUPUS-LIKE ANTICOAGULANT CORRECTED BY INTRAVENOUS IMMUNOGLOBULIN

We are reporting on a 47-year-old man who presented with a prolongatio n of the activated partial thromboplastin time (APTT) prior to orthope dic surgery. An evaluation suggested an inhibitor when his plasma prol onged a normal control APTT upon 50:50 solution of patients with norma l plasma. The platelet-neutralizing procedure (PNP), anticardiolipin a ntibody, and antinuclear antibody (ANA) were positive. Further studies revealed decreased von Willebrand factor ristocetin cofactor (vWF:RCo F), von Willebrand factor antigen (vWF:Ag), an inhibitor to vWF, and a bsent high-molecular-weight vWF multimeters. Assays of FVIII:C, FIX, a nd FXI were nonparallel to the standard curve. Intravenous immunoglobu lin (IVIG) corrected the APTT, multimeric pattern, and FVIII:C by the 7th day postinfusion. This case demonstrates the efficacy of IVIG for acquired von Willebrand's syndrome (vWS) and also represents a unique combination of a lupus-like anticoagulant and acquired vWS in a patien t without the full serological requirement for systemic lupus erythema tosus (SLE). Whether patients with acquired vWS and lupus inhibitors a re more or less susceptible to either a thrombotic complication or hem orrhage is not established. Prospective studies for the incidence of l upus inhibitor/antiphospholipid syndromes and VWF deficiencies are nee ded to assess this question. (C) 1994 Wiley-Liss, Inc.