Acquired Bernaud-Soulier syndrome: A case with necrotizing vasculitis and thrombosis

We describe a patient with positive antinuclear antibodies, polyclonal gamm opathy and high level of circulating immunocomplexes, resulting in vascular purpura. In addition, the patient had a slightly prolonged bleeding time a nd an isolated defect of ristocetin-induced platelet aggregation (RIPA) in platelet-rich plasma (PRP). The patient's plasma also inhibited RIPA in nor mal PRP and in normal platelet suspension. The activity and multimeric stru cture of plasmatic von Willebrand factor showed no alteration. We could dem onstrate an autoantibody against platelet membrane glycoprotein (GP) Ib, us ing an ELISA-type assay. These data suggest an acquired Bernard-Soulier syn drome. We suggest that the patient had an immunocomplex-mediated leukocytoc lastic vasculitis accompanied by production of antinuclear autoantibodies a s well as the presence of an autoantibody against GPlb. The titer of the an ti-GPlb antibody, however, was too low to induce significant platelet-type bleeding tendency, only laboratory alterations were found. Moreover, in a l ater stage of her disease, she developed a severe necrotizing vasculitis wh ich was followed by a deep venous thrombosis. Copyright (C) 2000 S. Karger AG, Basel.