Acquired hypolipoproteinemia.
We present a six-year follow-up of a boy with a novel type of hypolipoproteinemia, with clinical and biochemical features distinct from classical hypoalphalipoproteinemias. There were abnormally low concentrations of total and high-density lipoprotein (HDL) cholesterol, apolipoprotein (apo) B, apo A-I, and apo A-II, and the phospholipids were decreased. The most striking abnormality was an extra fraction containing mainly phospholipids and apo A-I in the HDL3 subfraction. This fraction is reminiscent of concentric 20- to 50-nm-diameter lamellar phospholipid liposomes. Plasma lecithin:cholesterol acyltransferase activity was strongly decreased. We noted a persisting polyclonal hypergammaglobulinemia, hematological abnormalities (hemolytic anemia and thrombocytopenia), and a progressive splenomegaly. After the five-year follow-up, the patient had recurrent severe infections; moderate hematuria and proteinuria developed gradually. Treatment with corticosteroids and immunoglobulins improved thrombocytopenia and hypolipoproteinemia. These clinical and biochemical findings differ from those in the known primary and secondary hypo-alpha-lipoproteinemia syndromes. Although investigation of the relatives suggests a familial predisposition for hypo-alpha-lipoproteinemia, the subject's condition can be regarded as acquired.
De Buyzere
M
M
Department of Clinical Chemistry, University Hospital, Gent, Belgium.
Delanghe
J
J
Labeur
C
C
Noens
L
L
Benoit
Y
Y
Baert
J
J
Rosseneu
M
M
D000293:Adolescent; D000743:Anemia, Hemolytic / Q000150:complications; D016632:Apolipoprotein A-I / Q000172:deficiency; D016633:Apolipoprotein A-II / Q000172:deficiency; D001055:Apolipoproteins B / Q000172:deficiency; D008076:Cholesterol, HDL / Q000172:deficiency; D006801:Humans; D007009:Hypolipoproteinemias / Q000097:blood / Q000150:complications / Q000175:diagnosis*; D008074:Lipoproteins / Q000097:blood; D008075:Lipoproteins, HDL / Q000097:blood / Q000648:ultrastructure; D053440:Lipoproteins, HDL3; D008297:Male; D010316:Particle Size; D007862:Phosphatidylcholine-Sterol O-Acyltransferase / Q000097:blood; D010743:Phospholipids / Q000097:blood; D013163:Splenomegaly; D013921:Thrombocytopenia / Q000150:complications
D016632:Apolipoprotein A-I; D016633:Apolipoprotein A-II; D001055:Apolipoproteins B; D008076:Cholesterol, HDL; D008074:Lipoproteins; D008075:Lipoproteins, HDL; D053440:Lipoproteins, HDL3; D010743:Phospholipids; D007862:Phosphatidylcholine-Sterol O-Acyltransferase