Background: The management involving genetic evaluation and therapy for SHTG of 21 subjects having SHTG either associated with AP or not is discussed within the context of a multinational, clinical case record analysis. Methods: A retrospective case series study of 21 patients with severe hypertriglyceridemia (SHTG) with or without acute pancreatitis (AP) involving recurrent AP cases was performed. The clinical profile, genetic background, and results of treatments using therapeutic plasma exchange (TPE) and/or exchange transfusion (ET) were analyzed among a mixed group of adult patients, children, pregnant females, and one neonate. Results: Patients were symptomatic and were admitted via the ER. Patients who received therapy using TPE or ET had a significant reduction in plasma lipid and lipoprotein levels with improvement in their symptoms. Other treatments, such as TG-lowering agents, can also be used but are less beneficial at this stage in the management of rapidly lowering TG levels.

Efficacy of plasma exchange and exchange transfusion in the clinical management of severe hypertriglyceridemia in emergency

Fornari, E;Mancioppi, V;Zenti, M G;Maffeis, C;
2026-01-01

Abstract

Background: The management involving genetic evaluation and therapy for SHTG of 21 subjects having SHTG either associated with AP or not is discussed within the context of a multinational, clinical case record analysis. Methods: A retrospective case series study of 21 patients with severe hypertriglyceridemia (SHTG) with or without acute pancreatitis (AP) involving recurrent AP cases was performed. The clinical profile, genetic background, and results of treatments using therapeutic plasma exchange (TPE) and/or exchange transfusion (ET) were analyzed among a mixed group of adult patients, children, pregnant females, and one neonate. Results: Patients were symptomatic and were admitted via the ER. Patients who received therapy using TPE or ET had a significant reduction in plasma lipid and lipoprotein levels with improvement in their symptoms. Other treatments, such as TG-lowering agents, can also be used but are less beneficial at this stage in the management of rapidly lowering TG levels.
2026
Acute pancreatitis (AP)
Dyslipidemia
Exchange transfusion (ET)
Familial chylomicronemia syndrome (FCS)
Rare disease
Severe hypertriglyceridemia (SHTG)
Therapeutic plasma exchange (TPE)
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11562/1206967
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