European Journal of Molecular & Clinical Medicine

Abstract

Thalassemias are group of inherited autosomal recessive blood disorder. The genetic defect, which could be either mutation or deletion, results in complete absence or reduction synthesis of one of the globin chain that make up hemoglobin. This cause reduction of haemoglobin molecules production ,resulting in decreased of oxygen carrying capacity of the circulation thus causing anemia, the characteristic presenting symptom of the thalassemia. Good chelation therapy and regular blood transfusion protocol have increased the life expectancy.
Objectives : is to compare serum ferritin level and liver function test in a group of patients with beta thalassemia major in response to subcutaneous and oral chelating agent .
Patients and Methods: Prospective analytic study design done on group of 60 patients with beta thalassemia major diagnosed by hemoglobin electrophoresis registered in thalassemia center in AL – Zahra Teaching Hospital for Maternity and Children in AL- Najaf AL- Ashraf , during a period from
1st of February 2019 to 1st of February 2020, aged 2.5 to 17.9 years with serum ferritin levels above 1000 ng/ml and liver transaminases below 5 folds the normal upper limit .
The patients divided into 2 groups, 30 patients were on Deferasirox {Exjade} therapy and 30 patients were on Deferoxamine {Desferal} therapy .
Base line S. Ferritin value as well as LFT (S. GOT , S. GPT , ALP and TSB) value, were taken as base line level and then every (8-12) weeks and were used to assess the changes that occurred in response to subcutaneous and oral chelating agent .
Results : Our study show significant decline in SF (P value <0.05) in both groups after 1 year of treatment with oral or subcutaneous chelating agent. Patients on Exjade show more rate of decrement of serum ferritin (11%) compared to patients taken Desferal who a rate of decrement (6%). Patients on Exjade and Desferal therapy show increment in liver enzymes after 1 year of treatment with statistically significant results, p-value < 0.05 .
Conclusions
We found superiority in oral iron chelating agents (Exjade) to subcutaneous iron chelating agents (Desferal). Serum ferritin level is suitable for long term monitoring as an indicator of efficacy than liver biopsy .