[Type III hyperlipoproteinemia in monoclonal IgM gammopathy].
A 42-year-old woman, found to have increased blood-lipid levels, developed hand-line xanthomas 3 months later. She also had an increased erythrocyte-sedimentation rate and a type lambda monoclonal IgM abnormality. In the course of the following 4 years the IgM concentration rose (from 5.8 to 12.3 g/l) steadily and synchronously with those of the triglycerides (from 147 to 391 mg/dl) and cholesterol (from 212 to 380 mg/dl). During the entire period of observation the ratio of VLDL cholesterol and triglyceride concentrations was elevated (greater than 0.4; normal: less than 0.3). Division of the lipoprotein fractions pointed to a type III hyperlipoproteinaemia. She also had an abnormality of lipid metabolism on the basis of a genetic defect (apolipoprotein-E2 homozygotism), which only manifested itself when an additional factor, IgM paraproteinaemia, was present. Paraproteins thus apparently interfered with the breakdown of the lipoproteins. Neither bezafibrate in increasing doses (200-800 mg daily) with low-fat diet nor administration of lovastatin (20 mg twice daily) together with nicotinic acid (500 mg twice daily) were adequate treatment for this form of auto-immune hyperlipidaemia.
Abteilung Innere Medizin III, Universität Heidelberg.
R
R
Hambrecht
E
E
von Hodenberg
G
G
Schuler
K
K
Tischbirek
W
W
Kübler
D000328:Adult; D001629:Bezafibrate / Q000627:therapeutic use; D002784:Cholesterol / Q000097:blood; D004041:Dietary Fats / Q000627:therapeutic use; D005260:Female; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications*; D006952:Hyperlipoproteinemia Type III / Q000097:blood / Q000209:etiology* / Q000628:therapy; D007075:Immunoglobulin M / Q000032:analysis; D007146:Immunoglobulin lambda-Chains; D008055:Lipids / Q000097:blood; D008148:Lovastatin / Q000627:therapeutic use; D009525:Niacin / Q000627:therapeutic use; D014280:Triglycerides / Q000097:blood
D004041:Dietary Fats; D007075:Immunoglobulin M; D007146:Immunoglobulin lambda-Chains; D008055:Lipids; D014280:Triglycerides; D009525:Niacin; D002784:Cholesterol; D008148:Lovastatin; D001629:Bezafibrate
Typ-III-Hyperlipoproteinämie bei monoklonaler IgM-Gammopathie.