title: Acquired Bernard-Soulier syndrome: a case with necrotizing vasculitis and thrombosis.
abstract: We describe a patient with positive antinuclear antibodies, polyclonal gammopathy and high level of circulating immunocomplexes, resulting in vascular purpura. In addition, the patient had a slightly prolonged bleeding time and an isolated defect of ristocetin-induced platelet aggregation (RIPA) in platelet-rich plasma (PRP). The patient's plasma also inhibited RIPA in normal PRP and in normal platelet suspension. The activity and multimeric structure of plasmatic von Willebrand factor showed no alteration. We could demonstrate an autoantibody against platelet membrane glycoprotein (GP) Ib, using an ELISA-type assay. These data suggest an acquired Bernard-Soulier syndrome. We suggest that the patient had an immunocomplex-mediated leukocytoclastic vasculitis accompanied by production of antinuclear autoantibodies as well as the presence of an autoantibody against GPIb. The titer of the anti-GPIb antibody, however, was too low to induce significant platelet-type bleeding tendency, only laboratory alterations were found. Moreover, in a later stage of her disease, she developed a severe necrotizing vasculitis which was followed by a deep venous thrombosis.
authors.affiliation: Second Department of Medicine, University Medical School, Debrecen, Hungary.
authors.forename: I
authors.initials: I
authors.lastname: Tornai
authors.forename: Z
authors.initials: Z
authors.lastname: Boda
authors.forename: A
authors.initials: A
authors.lastname: Schlammadinger
authors.forename: A
authors.initials: A
authors.lastname: Juhasz
authors.forename: N
authors.initials: N
authors.lastname: Cauwenberghs
authors.forename: H
authors.initials: H
authors.lastname: Deckmyn
authors.forename: J
authors.initials: J
authors.lastname: Harsfalvi
mesh_terms: D000974:Antibodies, Antinuclear / Q000097:blood / Q000494:pharmacology; D000911:Antibodies, Monoclonal / Q000494:pharmacology; D000941:Antigens / Q000097:blood / Q000494:pharmacology; D001323:Autoantibodies / Q000097:blood; D001606:Bernard-Soulier Syndrome / Q000150:complications* / Q000276:immunology*; D001760:Bleeding Time; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006801:Humans; D008875:Middle Aged; D010949:Plasma / Q000187:drug effects / Q000276:immunology; D010974:Platelet Aggregation / Q000187:drug effects; D010979:Platelet Function Tests / Q000295:instrumentation; D019038:Platelet Glycoprotein GPIb-IX Complex / Q000276:immunology; D010488:Polyarteritis Nodosa / Q000276:immunology; D012310:Ristocetin / Q000494:pharmacology; D020246:Venous Thrombosis / Q000276:immunology; D014841:von Willebrand Factor / Q000276:immunology / Q000494:pharmacology
chemical_list: D000974:Antibodies, Antinuclear; D000911:Antibodies, Monoclonal; D000941:Antigens; D001323:Autoantibodies; D019038:Platelet Glycoprotein GPIb-IX Complex; D014841:von Willebrand Factor; D012310:Ristocetin