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A non‐thrombocytopenic bleeding disorder due to an IgG4‐kappa anti‐GPIIb/IIIa autoantibody
Author(s) -
McMillan R.,
Bowditch R. D.,
Tani P.,
Anderson H.,
Goodnight S.
Publication year - 1996
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1046/j.1365-2141.1996.d01-1957.x
Subject(s) - autoantibody , medicine , platelet , immunology , thrombocytopenic purpura , antibody , fibrinogen , platelet membrane glycoprotein , kappa , linguistics , philosophy
Autoantibodies in chronic immune thrombo‐cytopenic purpura occasionally interfere with platelet function. We describe a patient with a normal platelet count who had clinically significant mucosal bleeding, a prolonged bleeding time and abnormal platelet aggregation. The patient had high titres of an IgG4 kappa autoantibody, directed to a cation‐dependent epitope on platelet glycoprotein IIb/IIIa, which blocked the binding of fibrinogen and fibronectin to this complex. Corticosteroid treatment resulted in clinical improvement and a marked drop in autoantibody concentration. The lack of thrombocytopenia in this patient, despite high autoantibody levels, is best explained by the poor recognition of IgG4 antibodies by phagocytic cells.