Indications for apheresis as an ultima ratio treatment of refractory hyperlipidemias

P Grützmacher,C Kleinert,C Dorbath,B Öhm

doi:10.1007/s11789-015-0070-4

Abstract

Lipid apheresis is at present well established in routine treatment of diverse hyperlipoproteinemias refractory to conventional dietary and medical regimens, especially in countries with high medical and socioeconomic standards. Severe familial hypercholesterolemia with atherosclerotic vessel disease involving the coronary arteries is the most frequent indication for lipid apheresis as well as homozygous familial hypercholesterolemia before the development of cardiovascular complications.In hyperlipoproteinemia (a) with progressive vessel disease, lipid apheresis is regularly accepted in Germany. The indication of apheresis in Refsum’s disease and the chylomicronemia syndrome is described.

Highlights

During the past 50 years, apheresis therapy has been applied successfully in patients with different disturbances of lipid metabolism
E.g., the chylomicronemia syndrome, apheresis is performed as an emergency treatment in hospitalized patients; in other diseases, e.g., refractory hypercholesterolemia and hyperlipoproteinemia (a), maintenance apheresis therapy is required, usually on an ambulatory basis
As most apheresis techniques have been invented in Japan and Germany, it is understandable that lipid apheresis techniques are applied there frequently in experimental studies as well as in clinical routine practice

Summary

Introduction

During the past 50 years, apheresis therapy has been applied successfully in patients with different disturbances of lipid metabolism. E.g., the chylomicronemia syndrome, apheresis is performed as an emergency treatment in hospitalized patients; in other diseases, e.g., refractory hypercholesterolemia and hyperlipoproteinemia (a), maintenance apheresis therapy is required, usually on an ambulatory basis. Plasma exchange has been the sole apheresis technique for more than 10 years for all disorders and is still frequently used in the chylomicronemia syndrome. Lipid apheresis treatment except from emergency cases is indicated only in case of insufficient dietary and medical treatment. Because of the high costs and technical efforts, clinical use of lipid apheresis is mainly influenced by the economic prosperity of the social and health care system, which enables broad routine use only in industrialized countries. In Germany, e.g., health insurances are generally obliged to cover the costs of ambulatory treatment in patients with hypercholesterolemia and hyperlipoproteinemia (a) with progressive cardiovascular disease refractory to optimal dietary and medical treatment. For in-hospital treatment, especially for the use of apheresis in emergencies, such appointments are usually not necessary

Dietary and medical therapy

Potential role of new drugs

Findings

Severe hypertriglyceridemia