Type III Hyperlipoproteinemia
Helen Liapis, MD
Joseph Gaut, MD, PhD
Key Facts
Etiology/Pathogenesis
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APOE2 homozygotes
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Autosomal recessive
Clinical Issues
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Males more commonly affected
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Nephrotic syndrome
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Premature vascular disease
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Palmar and tuberous xanthomas
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Hypercholesterolemia (> 300 mg/dL)
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VLDL/TG ratio > 0.3
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Hypertriglyceridemia (> 300 mg/dL)
Microscopic Pathology
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Glomeruli
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Foam cells
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Segmental sclerosis
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Top Differential Diagnoses
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Lipoprotein glomerulopathy
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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
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Type III hyperlipidemia
Definitions
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Hypercholesterolemia and hypertriglyceridemia associated with apolipoprotein-E2 allele
ETIOLOGY/PATHOGENESIS
Genetics
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APOE
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Encodes apolipoprotein E
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