Successful Treatment of Homozygous Familial Hypercholesterolemia Using Cascade Filtration Plasmapheresis


KARDAŞ F., ÇETİN A., Solmaz M., Büyükoǧlan R., KAYNAR L., Kendirci M., ...More

TURKISH JOURNAL OF HEMATOLOGY, vol.29, no.4, pp.334-341, 2012 (SCI-Expanded) identifier identifier

  • Publication Type: Article / Article
  • Volume: 29 Issue: 4
  • Publication Date: 2012
  • Doi Number: 10.5152/tjh.2011.20
  • Journal Name: TURKISH JOURNAL OF HEMATOLOGY
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus, TR DİZİN (ULAKBİM)
  • Page Numbers: pp.334-341
  • Keywords: Familial hypercholesterolemia, Cascade filtration plasmapheresis, Low-density lipoprotein cholesterol (LDL-C)
  • Istanbul Medipol University Affiliated: No

Abstract

Objective: The aim of this study was to report the efficacy of low-density lipoprotein cholesterol (LDL-C) apheresis using a cascade filtration system in pediatric patients with homozygous familial hypercholesterolemia (FH), and to clarify the associated adverse effects and difficulties. Material and Methods: LDL-C apheresis using a cascade filtration system was performed in 3 pediatric patients with homozygous FH; in total, 120 apheresis sessions were performed. Results: Cascade filtration therapy significantly reduced the mean LDL-C values from 418 ± 62 mg/dL to 145 ± 43 mg/ dL (p= 0.011). We observed an acute mean reduction in the plasma level of total cholesterol (57.9%), LDL-C (70.8%), and high-density lipoprotein cholesterol (HDL-C) (40.7%). Treatments were well tolerated. The most frequent clinical adverse effects were hypotension in 3 sessions (2.5%), chills (1.7%) in 2 sessions, and nausea/vomiting in 3 sessions (2.5%). Conclusion: Our experience using the cascade filtration system with 3 patients included good clinical outcomes and laboratory findings, safe usage, and minor adverse effects and technical problems.