ORAK HÜCRELİ ANEMİ 

Tanım: Orak hücre sendromları HbS ile beraber olan bir grup hastalığı tanımlamaktadır. Orak hücre anemisi ise HbS‘in homozigot Şeklini tanımlar. HbS, beta zincirinin NH2 ucunda 6.amino asidi olan Glutamin yerine Valin geçmesi ile yani baz düzeyinde GAG yerine GTG gelmesi ile oluşur. 

Gerekçe: Hiperviskosite ve volüm yüklemesini önlemek amacı ile özellikle akut iskemik inmede, ağrı krizinde, akut göğüs sendromunda, akut hepatik krizde ve yaşamsal veya organ kaybı riski olan hastalarda eritrositaferez tercih edilir. 

İşlem: Eritrositaferez

Sıklığı: 

 Akut olgularda Hb S düzeyini <%30‘u sağlamak için bir işlem 

 Kronik olgularda Hb S düzeyini <%30‘u sağlamak için 3-4 haftada bir, <%50‘i sağlamak için 4-5 haftada bir 

Replasman Sıvısı: Hemoglobin S negatif lökosit azaltılmış eritrosit, mümkünse en azından E, C ve Kell Ag‘leri ile uyumlu olmalı. 

Uygulama sayı ve/veya süresi: Akut atakta bir seans, kronik ağrıda kısa süreli birkaç seans ve inme profilaksisinde ömür boyu tercih edilmelidir. 

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