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Acquired hypolipoproteinemia.

M De Buyzere1, J Delanghe, C Labeur

  • 1Department of Clinical Chemistry, University Hospital, Gent, Belgium.

Clinical Chemistry
|May 1, 1992
PubMed
Summary
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This study details a unique form of hypolipoproteinemia in a boy, characterized by low lipid levels and unusual HDL3 fractions. Treatment improved his blood lipid profile and reduced thrombocytopenia.

Area of Science:

  • Biochemistry
  • Genetics
  • Clinical Medicine

Background:

  • Hypolipoproteinemias are a group of disorders characterized by low levels of lipids and lipoproteins in the blood.
  • Classical hypoalphalipoproteinemias are associated with low levels of high-density lipoprotein (HDL) cholesterol and apolipoprotein A-I (apo A-I).

Observation:

  • A boy presented with a novel type of hypolipoproteinemia, distinct from classical forms, over a six-year follow-up.
  • Biochemical analysis revealed abnormally low total and HDL cholesterol, apolipoprotein B (apo B), apo A-I, apo A-II, and decreased phospholipids.
  • A unique abnormality was an extra fraction in the HDL3 subfraction, containing phospholipids and apo A-I, resembling lamellar liposomes.

Findings:

  • Plasma lecithin:cholesterol acyltransferase (LCAT) activity was significantly decreased.

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  • The patient exhibited persistent polyclonal hypergammaglobulinemia, hemolytic anemia, thrombocytopenia, and progressive splenomegaly.
  • Recurrent infections, hematuria, and proteinuria developed over time.

    • Implications:

    • Treatment with corticosteroids and immunoglobulins led to improvements in thrombocytopenia and hypolipoproteinemia.
    • The distinct clinical and biochemical profile differentiates this condition from known primary and secondary hypo-alpha-lipoproteinemia syndromes.
    • While a familial predisposition is suggested, the patient's condition is considered acquired.