Therapeutic Plasma Exchange in Patients with Chylomicronemia Syndrome Complicated by Acute Pancreatitis

Chylomicronemia syndrome (CMS) is a rare disorder characterized by the presence of chylomicrons in the fasting state causing a milky appearance of plasma, eruptive xanthomas, and hepatosplenomegaly; an acute and potentially life threatening complication is severe acute pancreatitis. The underlying d...

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Bibliographic Details
Published in:Therapeutic apheresis 1999-08, Vol.3 (3), p.227-233
Main Authors: Lennertz, A, Parhofer, K G, Samtleben, W, Bosch, T
Format: Article
Language:English
Subjects:
Acute Disease
Adult
Chylomicronemia syndrome
Chylomicrons
Chylomicrons - blood
Female
Humans
Hypertriglyceridemia
Lipid Metabolism, Inborn Errors - therapy
Male
Middle Aged
Pancreatitis
Pancreatitis - etiology
Particle Size
Plasma Exchange
Retrospective Studies
Syndrome
Therapeutic plasma exchange
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Summary:Chylomicronemia syndrome (CMS) is a rare disorder characterized by the presence of chylomicrons in the fasting state causing a milky appearance of plasma, eruptive xanthomas, and hepatosplenomegaly; an acute and potentially life threatening complication is severe acute pancreatitis. The underlying defects are inborn errors of metabolism such as deficiencies of lipoprotein lipase (LPL) or apoprotein C‐II (apo C‐II) as well as familial hypertriglyceridemia. Moreover, CMS can be precipitated when mild hypertriglyceridemia is exacerbated by additional factors such diabetes mellitus, ethanol abuse, or pregnancy. The purpose of the present study was to retrospectively analyze the results of therapeutic plasma exchange (TPE) in 5 patients transferred to our hospital for severe acute pancreatitis due to chylomicronemia syndrome. In a total of 7 TPE sessions, on average 3,286 ± 247 ml of plasma (i.e., about 1 patient plasma volume) were treated per session. Triglyceride (TG) levels were decreased from 4,972 ± 2,469 mg/dl on admission to 1,614 ± 1,276 mg/dl (−70%) after the TPE sessions, and a further decrease was achieved by conservative treatment. Part of the TG reducing effect of the treatment was probably due to heparin induced lipolysis. Acute pancreatitis was resolved in all cases, and 1 pregnant patient delivered without problems at term. In summary, 1 or 2 TPE sessions sufficed to substantially decrease the bulk of triglycerides in acutely exacerbated chylomicronemia syndrome causing a rapid resolution of acute severe pancreatitis.
ISSN:1091-6660
1526-0968
DOI:10.1046/j.1526-0968.1999.00158.x