The Challenge of Unresolving Chylomicronemia and Recurring Pancreatitis: Lessons Learned on the Treatment of Severe Hypertriglyceridemia

Severe hypertriglyceridemia (HTG) in the chylomicronemia (CM) range is associated with acute pancreatitis. Plasma exchange (PEX) has been used for the treatment of chronic CM in the setting of recurrent pancreatitis. We present a patient with multifactorial CM syndrome and recurrent pancreatitis recently started on biweekly sessions of PEX. We share lessons learned on the management of multifactorial CM syndrome and highlight the potential complications associated with chronic apheresis.

Case Description:

A 37-year-old woman with a history of triglyceride (TG)-induced pancreatitis presents to the hospital with nausea and vomiting a week after an episode of diabetic ketoacidosis. The patient had two previous admissions for acute pancreatitis, an initial one 3 years prior and the most recent one, complicated by formation of a pseudocyst, 2 years later, in addition to multiple acute care visits for self-limited nausea, vomiting and abdominal pain. There was no evidence of acute pancreatitis on her current or most recent admission; however, TG remained consistently >1,575 mg/dL. For the past five years the patient has had severe HTG with occasional readings above 10,000 mg/dL She reports weight gain (current body mass index 31 kg/m²) and onset of type 2 diabetes after the birth of her youngest child 5 years ago. Outpatient medications include insulin, metformin (last hemoglobin A1c of 10.2%), fenofibrate and omega 3 ethyl esters. She discontinued alcohol intake after initial presentation, but otherwise follows no dietary restrictions. She was started on biweekly sessions of PEX two months prior to admission. During admission she was found to have catheter-associated bacteremia with septic embolization and development of a superior vena cava thrombus with extension into the right atrium. The central venous catheter used for PEX was removed and antibiotics and systemic anticoagulation initiated. After a 25-day hospital stay, she left to a long-term treatment facility. During hospitalization, the patient was actively treated with subcutaneous insulin and a diet low in fat and simple carbohydrates, with no PEX. Her TG level was 261 mg/dL at the time of discharge.

Discussion:

Multifactorial CM syndrome may present with extreme elevations of TG levels and recurrent pancreatitis but responds well to strict dietary changes and modification of underlying factors. Adherence to a diet low in fat and simple carbohydrates, together with management of poor diabetes control in this patient resulted in a significant improvement of her severe HTG. The use of chronic PEX in multifactorial CM syndrome is of unproved benefit and burdens patients with the risk of serious life-threatening complications.