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.