title: Intensive, long-term plasma exchange therapy for severe hypertriglyceridemia in acquired generalized lipoatrophy.
abstract: We report dramatic improvement in clinical and laboratory parameters after intensive plasma exchange therapy in a 15-yr-old girl with acquired generalized lipoatrophy and refractory hypertriglyceridemia. One hundred and twenty-five procedures were performed over 720 d. Two or three plasma volumes were exchanged per procedure, using peripheral venous access and albumin as replacement solution. Regression of painful cutaneous xanthomata and reduction in massive hepatomegaly were noted within the first two procedures. Triglyceride levels started at 109 mmol/liter (9670 mg/dl) and decreased acutely by 60-85%/procedure. Lipid removal averaged 83 g/procedure and was highly correlated with preexchange lipid levels. Lipid levels rebounded to baseline values within 7 d after exchange and appeared to rebound more rapidly after larger exchanges. Maximum benefit was achieved with weekly 1.5- to 2.0-volume exchanges. No significant decrease in apolipoprotein CII levels was detected after plasma exchange regardless of the volume of exchange; however, other plasma factors regulating triglyceride synthesis or clearance may have been removed during the procedures. Plasma exchange was well tolerated, without clinical, immunological, or hormonal deterioration. These data indicate that intensive plasma exchange therapy over a protracted time may produce sustained benefit in patients with severe, symptomatic hypertriglyceridemia refractory to standard medical therapy.
authors.affiliation: Department of Transfusion Medicine, Warren Magnuson Clinical Center, National Institutes of Health, Bethesda, Maryland 20892, USA.
authors.forename: Charles
authors.initials: C
authors.lastname: Bolan
authors.forename: Elif Arioglu
authors.initials: EA
authors.lastname: Oral
authors.forename: Phillip
authors.initials: P
authors.lastname: Gorden
authors.forename: Simeon
authors.initials: S
authors.lastname: Taylor
authors.forename: Susan F
authors.initials: SF
authors.lastname: Leitman
mesh_terms: D000273:Adipose Tissue / Q000473:pathology*; D000293:Adolescent; D001786:Blood Glucose / Q000032:analysis; D005230:Fatty Acids, Nonesterified / Q000097:blood; D005260:Female; D005528:Foot; D006801:Humans; D015228:Hypertriglyceridemia / Q000097:blood / Q000473:pathology / Q000628:therapy*; D007866:Leg; D008099:Liver / Q000201:enzymology / Q000473:pathology; D010951:Plasma Exchange; D011788:Quality of Life; D012871:Skin Diseases / Q000473:pathology / Q000628:therapy; D000637:Transaminases / Q000097:blood; D014280:Triglycerides / Q000097:blood; D014973:Xanthomatosis / Q000473:pathology / Q000628:therapy
chemical_list: D001786:Blood Glucose; D005230:Fatty Acids, Nonesterified; D014280:Triglycerides; D000637:Transaminases