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Bleeding is a rare manifestation of lupus anticoagulant-antiphospholipid syndrome unless associated with coagulation factor deficiency, thrombocytopenia, or intrinsic vascular defect. The authors report the clinical and laboratory findings in a 16-year-old boy with potent lupus anticoagulant who initially presented with recurrent epistaxis, hematuria, and gastrointestinal bleeding. Lupus anticoagulant potently inhibited assay systems for coagulation factors, but levels of factors II, IX, and XI appeared to be decreased (2-5% of mean normal levels). Within 2 weeks after diagnosis, spontaneous subdural hematomas developed. During hemostatic therapy, including plasmapheresis and infusions of recombinant activated factor VII and activated prothrombin complex concentrate, an ischemic stroke developed. Subsequent multifocal recurrent ischemic strokes developed despite immunosuppression. This case shows that lupus anticoagulant or antiphospholipid antibodies can cause both hemorrhagic and thrombotic complications in the same patient and may, in some patients, have multiple target antigens (eg, coagulation factors II, IX, XI).

Purpose: We report that treatment of an immune mediated postoperative Factor V (FV) deficiency with intravenous immune globulin (IVIg) resulted in serological and clinical disappearance of the inhibitor., Patients and Methods: A 9-year-old girl was exposed to bovine thrombin during cardiovascular surgery and subsequently developed severe, refractory hemorrhage caused by acquired FV deficiency (FV activity < 5%). Despite blood product transfusions, hemorrhage continued, and the patient was given IVIg, 400 mg/kg daily, for 9 day., Results: Prolonged clotting times immediately trended toward normal, and the hemorrhage ceased by the fifth IVIg treatment day, concomitant with increasing plasma FV activity and disappearance of human FV inhibitor activity. The patient's plasma initially had a much higher inhibitor titer against bovine FV (122-215 Bethesda units) than against human FV (3-4 Bethesda units). Circulating antibodies (IgM and IgG) to bovine and human thrombin and FV were detected by enzyme-linked immunosorbent assay (ELISA). After completion of IVIg treatment, IgG antibodies to bovine FV and thrombin persisted, as did high-titer inhibition of bovine FV, whereas the subpopulation of IgG and IgM antibodies reactive with human FV were undetectable., Conclusions: The inhibitor likely developed from a heterogenetic immune response to bovine FV contaminating the topical thrombin preparation used during surgery. To our knowledge, this is the first demonstration of immunological clearance of an acquired FV antibody associated with the use of IVIg. The data suggest an antiidiotypic mechanism of IVIg in modulating clearance of antihuman FV antibodies.