Type III hyperlipoproteinemia with xanthomas and multiple myeloma
Nancy J Burnside1, Lauren Alberta, Leslie Robinson-Bostom
1Department of Dermatology, Brown Medical School, Providence, Rhode Island 02903, USA.
Journal of the American Academy of Dermatology
|October 18, 2005
Summary
This case study highlights a rare instance where acquired Type III hyperlipoproteinemia was linked to IgA myeloma, with IgA antibodies impairing very low-density lipoprotein (VLDL) metabolism. Treatment of the myeloma led to improved lipid levels and xanthomas.
Area of Science:
- Lipid Metabolism Disorders
- Onco-immunology
- Genetic Dyslipidemia
Background:
- Type III hyperlipoproteinemia (HLPP) typically stems from inherited apolipoprotein E defects, increasing atherosclerosis risk.
- Acquired forms of HLPP are rare, occasionally linked to myeloma and immunoglobulin-lipoprotein complexes.
Observation:
- A 72-year-old man presented with refractory dyslipidemia, palmar crease, and tuberous xanthomas, despite prior well-controlled hypercholesterolemia.
- Diagnosis of Type III hyperlipoproteinemia was confirmed, with nonclassic defective apolipoprotein E identified.
- Malignancy workup revealed monoclonal IgA gammopathy; immunostaining showed IgA antibodies complexed with very low-density lipoprotein (VLDL).
Findings:
- IgA-VLDL complexes caused significant impairment of VLDL metabolism, leading to refractory dyslipidemia.
- The patient's apolipoprotein E genotype (E2/E3) was atypical for heritable Type III HLPP (usually E2/E2).
- Conventional Type III HLPP therapies were ineffective, suggesting an alternative underlying cause.
Implications:
- This case suggests a causal link between IgA myeloma and acquired Type III hyperlipoproteinemia, challenging the typical understanding of HLPP.
- Effective treatment involved chemotherapy for myeloma, leading to decreased IgA, improved lipid profiles, and resolution of xanthomas.
- Highlights the importance of investigating underlying malignancies in refractory dyslipidemia cases, especially those with unusual lipoprotein profiles.
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