ISSN: 1300-7777 E-ISSN: 1308-5263
Successful Treatment Of Homozygous Familial Hypercholesterolemia Using Cascade Filtration Plasmapheresis [Turk J Hematol]
Turk J Hematol. 2012; 29(4): 334-341 | DOI: 10.5152/tjh.2011.20  

Successful Treatment Of Homozygous Familial Hypercholesterolemia Using Cascade Filtration Plasmapheresis

Fatih Kardas1, Aysun Cetin2, Musa Solmaz3, Ruksan Buyukoglan3, Leylagul Kaynar3, Mustafa Kendirci1, Bulent Eser3, Ali Unal3
1Department Of Pediatric Metabolism, Erciyes University, School Of Medicine, Kayseri, Turkey
2Department Of Biochemistry, Erciyes University, School Of Medicine, Kayseri, Turkey
3Department Of Hematology, Erciyes University, School Of Medicine, Kayseri, Turkey

OBJECTIVE: The aim of our study is to discuss the efficacy of low-density lipoprotein-cholesterol (LDL-C) apheresis procedure using the cascade filtration system for pediatric patients with homozygous familial hypercholesterolemia (FH), and to clarify the adverse effects and difficulties.
METHODS: LDL apheresis using the cascade filtration system was performed in 3 pediatric patients with homozygous FH. In total, 120 apheresis sessions were performed for all patients.
RESULTS: Cascade filtration therapy significantly reduced the mean LDL-C values from 418 ± 62 mg/dl to 145 ± 43 mg/dl (p<0.05). We determined an acute mean reduction in the plasma levels of total cholesterol (57.9%), LDL cholesterol (70.8%), and high-density lipoprotein (HDL) cholesterol (40.7%). Treatments were well tolerated. The most frequent clinical adverse effects were hypotension in 3 sessions (2.5%), chills/feeling cold (1.7%) in 2 sessions, and nausea and vomiting in 3 sessions (2.5%).
CONCLUSION: Our experience with three patients using the cascade filtration system were, good clinical outcomes, laboratory findings, safety of usage, minor adverse effects and technical problems.

Keywords: Familial hypercholestrolemia, Cascade filtration plasmapheresis, Low density lipoprotein-cholesterol (LDL-C)


Homozigot Ailevi Hiperkolesterolemi’nin 'Cascade Filtration' Plazmaferez ile Başarılı Tedavisi

Fatih Kardas1, Aysun Cetin2, Musa Solmaz3, Ruksan Buyukoglan3, Leylagul Kaynar3, Mustafa Kendirci1, Bulent Eser3, Ali Unal3
1Erciyes Üniversitesi, Tıp Fakültesi, Pediatri Ana Bilim Dalı, Pediatrik Metabolizma Bilim Dalı, Kayseri
2Erciyes Üniversitesi, Tıp Fakültesi, Biyokimya Ana Bilim Dalı, Kayseri
3Erciyes Üniversitesi, Tıp Fakültesi, Iç Hastalıkları Ana Bilim Dalı, Hematoloji Bilim Dalı, Kayseri

AMAÇ: Çalışmamızın amacı, homozigot ailevi hiperkolesterolemili çocuk hastalarda ‘cascade filtration’ tekniği ile yapılan düşük dansiteli lipoprotein kolesterol (LDL-C) aferez tedavisinin etkinliğinin tespit edilmesi, işlem sırasında oluşabilecek yan etkilerin ve zorlukların ortaya konulmasıdır.
YÖNTEMLER: Homozigot ailevi hiperkolesterolemili 3 çocuk hastada, ‘cascade filtration’ sistemi kullanılarak LDL aferezi uygulandı. Tüm hastalar için toplam 120 aferez seansı yapıldı.
BULGULAR: ‘Cascade filtration’ tedavisi ile ortalama LDL-C seviyeleri, 418 ± 62 mg/dl’den 145 ± 43 mg/dl’ye belirgin olarak düştü (p<0.05). Total kolesterol, LDL-kolesterol ve HDL-kolesterol’ün plazma seviyelerindeki akut ortalama düşüş sırasıyla, %57.9, %70.8, %40.7 olarak bulundu. Tedaviler iyi tolare edildi. Klinik olarak en sık görülen yan etkiler, 3 seansda hipotansiyon (%2,5), 2 seansda titreme/üşüme (%1,7) ve 3 seansda bulantı/kusma (%2,5) idi.
SONUÇ: ‘Cascade filtraton’ sistemi ile 3 hastamızın tedavileri sonucunda, yüz güldürücü klinik bulgular ve laboratuvar sonuçları, güvenli kullanım, yok denecek kadar az yan etki ve teknik problemler tespit edilmiştir.

Anahtar Kelimeler: Ailevi hiperkolesterolemi, ‘cascade filtration’ plazmaferez, düşük dansiteli lipoprotein-kolesterol (LDL-C)


Fatih Kardas, Aysun Cetin, Musa Solmaz, Ruksan Buyukoglan, Leylagul Kaynar, Mustafa Kendirci, Bulent Eser, Ali Unal. Successful Treatment Of Homozygous Familial Hypercholesterolemia Using Cascade Filtration Plasmapheresis. Turk J Hematol. 2012; 29(4): 334-341

Corresponding Author: Fatih Kardas, Türkiye


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