A 50-year old male patient with familial hypercholesterolemia and hyperlipoproteinemia (a), who underwent low density lipoprotein-apheresis treatment developed heparin-induced thrombocytopenia type II (HIT II). Because heparin is contraindicated in patients with HIT, an alternative LDL-apheresis system and modified anticoagulation regimen was necessary. Treatment was changed to a new system called DALI (direct adsorption of lipids). After confirmation of the diagnosis HIT II, DALI LDL-apheresis was carried out with recombinant-hirudin (lepirudin) and citrate in order to prevent hypercoagulability. Efficient LDL-apheresis therapy with minimum therapeutic blood levels of lepirudin (1.4 mg/dl) was achieved with an initial intravenous bolus of 0.114 mg/kg of lepirudin followed by continuous lepirudin infusion of 0.350 mg/h. Thrombin-antithrombin III complex production was well controlled and other hemostatic markers showed no abnormalities. LDL-cholesterol and lipoprotein(a) concentrations were effectively reduced. R-hirudin offers a novel anticoagulation strategy and is, at present, the only alternative for patients with HIT II requiring LDL-apheresis on a regular basis.