Severe Hypertriglyceridemia (HTG) is associated with complications such as acute pancreatitis (AP) with high morbidity and mortality rates. We report a 42 years-old man with refractory HTG diagnosed at 19 years of age, and multiple episodes of AP, admitted with the suspicion of a new AP episode. Serum triglycerides were over 2000 mg/dl. His body mass index was 18 kg/m2, there was no evidence of xanthomas or xanthelasmas, but lipemia retinalis was found. Management included heparin and insulin, added to his usual treatment with fibrates, statins, omega-3 fatty acids, and orlistat. Due to lack of response, apheresis was started. After five sessions, triglycerides decreased to 588 mg/dl (82% reduction) and levels remained below 1000 mg/dl with daily apheresis. The patient continued with weekly sessions as outpatient with a sustained good response.
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