Plasma exchange in the treatment of heterozygous familial hypercholesterolaemia.
Five patients with heterozygous familial hypercholesterolaemia, whose response to diet and medication was inadequate, received additional treatment with plasma exchange (PE). Two and a half to three liters of plasma were replaced by a 5% albumin solution once every 3 weeks. Treatment with diet and cholestyramine was continued. This resulted in a reduction of serum total cholesterol (Tot-c) by 21.8-52.2% compared to pretreatment values. Normocholesterolaemia was not achieved. Since the mean Tot-c curve after PE exceeded the upper limit of the normal range approximately 10 days after exchange, the PE frequency was increased to once every 10 days. Tot-c was reduced by 42.6-57.5% and normocholesterolaemia was achieved in most cases. We conclude that normocholesterolaemia can be achieved by treatment which includes PE in all patients with heterozygous familial hypercholesterolaemia and inadequate response to the usual therapy. Individual adjustment of the PE frequency, based on the mean Tot-c curve after PE, is advised.