Type III Hyperlipoproteinemia
Helen Liapis, MD
Joseph Gaut, MD, PhD
Key Facts
Etiology/Pathogenesis
APOE2 homozygotes
Autosomal recessive
Clinical Issues
Males more commonly affected
Nephrotic syndrome
Premature vascular disease
Palmar and tuberous xanthomas
Hypercholesterolemia (> 300 mg/dL)
VLDL/TG ratio > 0.3
Hypertriglyceridemia (> 300 mg/dL)
Microscopic Pathology
Glomeruli
Foam cells
Segmental sclerosis
Top Differential Diagnoses
Lipoprotein glomerulopathy
FSGS
 Foamy macrophages  are seen in this glomerulus from biopsy of a 55-year-old man with nephrotic syndrome and type III hyperlipoproteinemia (cholesterol 559 mg/dL and triglycerides 2169 mg/dL). (Courtesy J. Churg, MD.) |
 Glomerular capillary lumen is obliterated by a mixture of foamy cells  , lipid vacuoles  , and dark-staining membrane-bound particles and lysosomes  . (Courtesy J. Churg, MD.) |
TERMINOLOGY
Synonyms
Type III hyperlipidemia
Definitions
Hypercholesterolemia and hypertriglyceridemia associated with apolipoprotein-E2 allele
ETIOLOGY/PATHOGENESIS
Genetics
APOE
Encodes apolipoprotein E
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