Antiapolipoprotein B cryoglobulinemia associated with normal plasma lipids and severe atherosclerosis.
A middle-aged female presented with diffuse planar xanthomas, associated with normal plasma lipids and a severe crescendo angina. Hematological testing disclosed the presence of a cryoglobulin binding to apolipoprotein B. The patient died after a short and dramatic clinical course. At autopsy, a markedly thickened aorta with diffuse atheromatous lesions of the major peripheral arteries were observed. Intracytoplasmic filaments (50-100 A), comparable to those described in amyloid deposits, were also detected. Endothelial cells were filled with lipid-containing vacuoles without extracellular lipid deposits. The reported case indicates that benign gammopathies against 'atherogenic' lipoprotein components, without clinical myeloma, may result in the development of severe arterial lesions.
D000906:Antibodies / Q000276:immunology*; D001013:Aorta, Thoracic / Q000473:pathology / Q000648:ultrastructure; D001055:Apolipoproteins B / Q000276:immunology*; D001161:Arteriosclerosis / Q000150:complications* / Q000175:diagnosis / Q000473:pathology; D003327:Coronary Disease / Q000209:etiology / Q000401:mortality; D003449:Cryoglobulinemia / Q000097:blood / Q000150:complications* / Q000473:pathology; D005260:Female; D006651:Histocytochemistry; D006801:Humans; D008055:Lipids / Q000097:blood*; D008854:Microscopy, Electron; D008875:Middle Aged
D000906:Antibodies; D001055:Apolipoproteins B; D008055:Lipids