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Transfusion. 2019 Sep 30. doi: 10.1111/trf.15532. [Epub ahead of print]

Efficacy of therapeutic plasma exchange in reducing the incidence of recurrent pancreatitis related to familial chylomicronemia.

Author information

1
Department of Pathology, Division of Transfusion Medicine, University of Texas Medical Branch, Galveston, Texas.
2
Department of Pediatrics, Division of Pediatric Endocrinology, University of Texas Medical Branch, Galveston, Texas.

Abstract

BACKGROUND:

Familial chylomicronemia syndrome (FCS) is caused by a genetic defect in triglyceride (TG) metabolism that leads to severe hypertriglyceridemia, which in turn is associated with multiple morbidities and may cause severe pancreatitis that is both recurrent and progressive. Management of hypertriglyceridemia in FCS is challenging, as both dietary and medical interventions are often ineffective. Therapeutic plasma exchange (TPE) has been shown to rapidly decrease circulating levels of chylomicrons and TGs in patients presenting with acute hypertriglyceridemic-associated pancreatitis. Conversely, limited evidence exists to suggest that prophylactic use of TPE is effective at preventing recurrence of acute pancreatitis.

CASE REPORT:

Herein, we report our experience with the use of chronic, prophylactic TPE to reduce the incidence of recurrent acute pancreatitis in a patient with FCS.

PMID:
31568575
DOI:
10.1111/trf.15532

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