Sequential Continuous Renal Replacement Therapy and Therapeutic Plasma Exchange in Pediatric Necrotizing Hypertriglyceridemia-Induced Pancreatitis with Suspected Familial Chylomicronemia Syndrome: A Case Report

Background: Hypertriglyceridemia-induced acute pancreatitis (HTG-AP) is a severe and underrecognized etiology of pancreatitis, accounting for 1% - 4% of all cases in adults, with extreme triglyceridemia raising the risk of fulminant multi-organ failure. When underlying lipoprotein lipase (LPL) deficiency is suspected—as in familial chylomicronemia syndrome (FCS)—conventional pharmacologic strategies are inherently limited, and therapeutic plasma exchange (TPE) represents the most effective currently available approach for the rapid removal of chylomicrons and triglyceride-rich lipoproteins from the circulation. Case Summary: We report a 14-year-old girl presenting with triglycerides > 14,000 mg/dL, severe necrotizing pancreatitis, acute kidney injury (AKI), mild acute respiratory distress syndrome (ARDS), and metabolic encephalopathy. No prior medical history was identified. Despite aggressive pharmacologic therapy with intravenous insulin, heparin, and fibrates, triglyceride levels remained critically elevated. Continuous renal replacement therapy (CRRT) was initiated for AKI, metabolic acidosis, and hemodynamic instability, achieving significant physiologic stabilization. However, because CRRT does not effectively clear chylomicrons, lipid levels remained dangerously high. Two urgent sessions of TPE reduced triglycerides from >14,000 mg/dL to approximately 300 mg/dL, with subsequent normalization of pancreatic enzymes and full recovery of renal, respiratory, and neurological function. The patient was discharged in good clinical condition. Conclusion: This case illustrates that CRRT and TPE fulfill distinct, non-overlapping roles in severe HTG-AP. CRRT provides essential metabolic bridge support, and TPE achieves definitive lipid clearance. When LPL deficiency is suspected, early escalation to TPE is potentially lifesaving. A high index of clinical suspicion and timely decision-making are the key determinants of outcome in this rare but catastrophic condition.