TRANSIENT ELASTOGRAPHY (TE) IS A USEFUL TOOL FOR ASSESSING THE RESPONSE OF LIVER IRON CHELATION IN SICKLE CELL DISEASE PATIENTS

Abstract

Sickle cell disease patients often need regular blood transfusions to improve both the quality of life and survival from the veno-occlusive complications of the disease. Deferasirox, a convenient long acting oral agent, has recently been introduced in clinical practice with promising efficacy.This study aims to evaluate the association of liver stiffness and possible fibrosis with iron deposition and confirm the use of elastography as a validated test of responding to chelation with low cost and easy access. 15 patients with sickle cell disease and systemic or occasional transfusionswere evaluated with MRI,transient elastography and biochemistry, for liver iron(LIC) and liver stiffness(LSM) before onset and one year after taking Deferasirox.All patients completed the study.Our results showed improvement in hepatic iron and hepatic stiffness after chelation therapy; Furthermore ALT, AST, LDH and ferritin levels have improved after 12 months of therapy with deferasirox.During the study no serious adverse events were encountered indicating the safety of the drug.Transient liver elastography findings correlate with serum ferritin and LIC in patients with sickle cell disease and it is a useful tool for assessing the response of liver iron chelation therapy.  Keywords: Sickle cell disease, iron chelation, transient elastography, transfusion, iron overload