January-March 2018

Study of Beta Thalassemia Major Patients with Special Reference To Iron Chelation and Cardiac Function

Ashka M. Shah1*, K.M.Meheriya2, CharulPurani3

1M. D. Paediatrics, 2Professor & Head, 3Assistant Professor, Department of Paediatrics, B. J. Medical College, Ahmedabad


BACKGROUND: Thalassemia is one of the most common monogenic disorder affecting human races. Generally an increase in body iron burden occurs in patients who are not receiving transfusions ranging from 2 to 5g/year. Iron overload cardiomyopathy is one of the common cause of death in Thalassemia major patients. AIMS AND OBJECTIVES: The purpose of this study is to study the cardiac abnormalities, efficacy and adverse effects of iron chelator agent- Deferasirox and to study the role of 2D echo and cardiac MRI in iron overload. MATERIALS AND METHODS: A prospective study of 185 patients was conducted from September 2013 to August 2015. All patients who were diagnosed as thalassemia majorwere included in my study and all patients> 10 years were subjected to cardiac evaluation while patients of age>12 years, having congenital heart disease or history of cardiac surgery sickle thalassemia, haemoglobin E disease, thalassemia intermedia and other thalassemia syndromes were excluded. OBSERVATIONS AND RESULTS:  In our present study regarding iron overload most common features were hepatomegaly and splenomegaly. Majority had their serum ferritin levels between 2500-5000µg/L. Out of 31 patients, 3 patients that got iron deposition on cardiac MRI, out of them two had normal systolic and diastolic function on ECHO. CONCLUSION: Serum ferritin can be used as an easily accessible serum marker for transfusion-induced iron overload.Cardiac MRI can be considered as a more sensitive indicator of cardiac iron overload than echocardiography.

Keywords: Iron overload features, Correlation between Serum ferritin and BTR, Dose Vs Response

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