Massive omental reticuloendothelial cell lipid uptake in Tangier disease after splenectomy.
Tangier disease is a rare, autosomal recessive condition characterized by cholesterol ester deposition in reticuloendothelial cells, abnormal chylomicron remnants, decreased low-density lipoprotein levels, and a marked deficiency of high-density lipoproteins. Apolipoprotein A-I, a major protein constituent of chylomicrons and high-density lipoproteins, has been shown to be structurally and metabolically abnormal in this disease (apolipoprotein A-ITangier). A 63-year-old Tangier homozygous man is described, who underwent splenectomy because of thrombocytopenia and splenomegaly. Subsequently, a large orange mass developed at the base of the mesentery, with several smaller omental masses and thickening of the entire omentum due to infiltration with lipid-laden macrophages. Splenectomy appears to predispose to such deposition, since such masses have not been observed in other Tangier homozygotes. The spleen appears to play a significant role in the removal of abnormal lipoproteins in Tangier homozygotes; therefore, splenectomy may be contraindicated in Tangier disease.
D001053:Apolipoproteins / Q000097:blood / Q000378:metabolism; D006099:Granuloma / Q000473:pathology; D006801:Humans; D007009:Hypolipoproteinemias / Q000378:metabolism*; D007728:Kupffer Cells / Q000648:ultrastructure; D050356:Lipid Metabolism; D008074:Lipoproteins / Q000097:blood; D008264:Macrophages / Q000378:metabolism* / Q000648:ultrastructure; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D009852:Omentum / Q000378:metabolism* / Q000473:pathology; D013156:Splenectomy / Q000009:adverse effects; D013631:Tangier Disease / Q000378:metabolism* / Q000473:pathology
D001053:Apolipoproteins; D008074:Lipoproteins