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<p>Aim. Chylomicronemia Syndrome (CS) refers to a group of lipid disorders characterized by the accumulation of chylomicrons in the plasma due to impaired lipolytic clearance. It encompasses various clinical features, including Familial Chylomicronemia Syndrome (FCS) and Multifactorial Chylomicronemia Syndrome (MCS). Diagnosing CS is a significant clinical challenge, as it is essential for identifying patients at highest risk of acute pancreatitis, preventing recurrence, and improving cardiovascular outcomes. Methods and Case Descriptions. Since 2008, we have identified eight patients residing in the same geographical area of Central Italy (Tuscany Region), all referred to us for severe and treatment-resistant hypertriglyceridemia. During this time, new therapeutic agents such as volanesorsen and lomitapide became available. However, none of these patients met the reimbursement criteria set by the Italian Medicines Agency (AIFA). We describe the clinical management and follow-up of these patients, in whom plasma and lipoprotein apheresis remain an effective therapy for reducing the risk of pancreatitis.</p>

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Experience with apheretic treatment in the chronic management of severe hypertriglyceridemia: case series

  • 期刊论文

  • 发表日期：

    2025年6月30日

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  • Francesco Sbrana,
  • Beatrice Dal Pino,
  • Carmen Corciulo,
  • Federico Bigazzi,
  • Tiziana Sampietro

摘要

Aim. Chylomicronemia Syndrome (CS) refers to a group of lipid disorders characterized by the accumulation of chylomicrons in the plasma due to impaired lipolytic clearance. It encompasses various clinical features, including Familial Chylomicronemia Syndrome (FCS) and Multifactorial Chylomicronemia Syndrome (MCS). Diagnosing CS is a significant clinical challenge, as it is essential for identifying patients at highest risk of acute pancreatitis, preventing recurrence, and improving cardiovascular outcomes. Methods and Case Descriptions. Since 2008, we have identified eight patients residing in the same geographical area of Central Italy (Tuscany Region), all referred to us for severe and treatment-resistant hypertriglyceridemia. During this time, new therapeutic agents such as volanesorsen and lomitapide became available. However, none of these patients met the reimbursement criteria set by the Italian Medicines Agency (AIFA). We describe the clinical management and follow-up of these patients, in whom plasma and lipoprotein apheresis remain an effective therapy for reducing the risk of pancreatitis.