The
direct thrombin inhibitor lepirudin is mainly applied in
heparin-induced
thrombocytopenia. We report here the case of a 37-year-old kurdish woman in whom Behcets disease was diagnosed in 1998 when she presented with a
Budd Chiari syndrome (BCS) complicated by
pulmonary embolism. Recurrent
venous thromboembolism (VTE) occurred despite
anticoagulant therapy with UFH,
LMWH or
phenprocoumon and various immunosuppressive therapy regimens. In 2001, when BCS recurred ultimately i.v.
lepirudin was administered. When the patient improved and remained clinically stable
lepirudin was applied subcutaneously. During long-term treatment with twice-daily 50 mg no further VTE was observed over the following years. Additionally, no
bleeding complications occurred. In May 2005
anticoagulant therapy was switched to
phenprocoumon. BCS reoccurred when INR values were suboptimal in February 2007, and
lepirudin treatment was immediately restarted. After admission the patient received 50 mg b.i.d.
lepirudin s.c. with plasma levels in the therapeutic range (0.5-1.0 mg / l). Over the following months,
lepirudin levels repeatedly exceeded the upper limit of this range and the dosage was stepwise reduced. Finally, 20 mg b.i.d. were sufficient to obtain therapeutic levels. Renal function was normal, but
lepirudin antibodies were present in high titer, as assessed by ELISA. We suppose that these
antibodies reduce renal filtration of
lepirudin thus leading to increased plasma levels. This case is an example for successful long-term therapeutic-dose anticoagulation with s.c.
lepirudin in a patient with Behcets disease and recurrent VTE despite therapeutic
anticoagulant therapy with
LMWH or
vitamin K antagonists. However, frequent measurement of
lepirudin plasma levels is needed. If stepwise dose lowering is required over time, the presence of
lepirudin antibodies should be considered.