Successful treatment of homozygous familial hypercholesterolemia using cascade filtration plasmapheresis Homozigot ailevi hiperkolesterolemi'nin 'cascade filtration' plazmaferezi ile başarılı tedavisi

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: Cascade filtration plasmapheresis, Familial hypercholesterolemia, 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.