# Outpatient monthly plasmapheresis with post-PLEX evinacumab in pediatric homozygous familial hypercholesterolemia: a case report on port access and immunoglobulin preservation

## TL;DR

This case report describes a new outpatient treatment combining plasmapheresis and evinacumab for a child with a rare cholesterol disorder.

## Contribution

First pediatric outpatient use of monthly plasmapheresis with post-PLEX evinacumab using dual-port access and immunoglobulin preservation.

## Key findings

- Monthly plasmapheresis with post-PLEX evinacumab was successfully implemented in a pediatric patient.
- Dual-port access and Octaplasma support enabled sustained therapy and preserved immunoglobulin levels.
- The case highlights the need for standardized protocols for combining evinacumab with extracorporeal treatments in children.

## Abstract

Homozygous familial hypercholesterolemia (HoFH) is a rare genetic disorder characterized by severely elevated low-density lipoprotein (LDL) cholesterol levels, predisposing patients to early cardiovascular disease. While LDL apheresis is the standard extracorporeal therapy, plasmapheresis (PLEX) is often used in younger children due to limitations in vascular access. Evinacumab, an ANGPTL3 inhibitor, has emerged as an effective adjunct for lowering LDL cholesterol. This case, to our knowledge, describes the first pediatric outpatient implementation of monthly plasmapheresis with post-PLEX Evinacumab infusions, enabled by dual-port access and Octaplasma support to maintain immunoglobulin levels. This report highlights procedural innovations that enabled sustained therapy and emphasizes the need for standardized approaches for combining Evinacumab with extracorporeal treatment in pediatric HoFH.