A Case of Budd-Chiari Syndrome with Lupus Anticoagulant

Abstract

A 34-year-old female with Budd-Chiari syndrome was admitted for the abnormal findings on hemostatic examinations to our clinic in 1983. PTT and aPTT were remarkablly prolonged and PT was slightly prolonegd, but whole blood clotting time and each coagulation factor were within normal range. The plasma from this patient showed prolonged kaolin clotting time (KCT) which failed to normalize with normal plasma, while the platelet rich plasma from her normalized normal KCT. Those findings were consistent with the presence of lupus anticoagulant. Further examination clarified that the anticoagulant activity from patient's plasma was found in the second peak on Sephadex G-200 chromatography, and reacted with phosphatidylserine but not with other phospholipids on immunodiffusion. we also demonstrated the accelerated genaration of procoagulant activity in a modified thromboplastin generation time test and prolongation of Kaolin-induced fibrinolytic activity indicating lowered intrinsic fibrinolytic activity. Those results might be associated with the thrombotic events in this patient.
Furthermore, immunological studies indicated increased IgG, positive anti-DNA antibody and anti-nuclear antibody, and positive direct Coombs' test.