Clinical, Hematological Profile and Status of Iron Overload in Children with Transfusion Dependent Thalassemia : A Study from Rural Northern India

Abstract

Thalassemias are one of most common type of haemolytic anaemia caused due to ineffective erythropoiesis requiring lifelong blood transfusions leading to chronic iron overload resulting in vasrious complications restricting the life span of most patients to 3rd-4th decade. Objective: To evaluate clinical, haematological profile and status of iron overload in patients of transfusion dependent thalassemia. Methods: We have enrolled 42 patients presenting to our centre for regular blood transfusion, the participants were followed up for a period of 1 year and samples were send at 3 monthly interval for CBC, Serum ferritin(S.F) , Serum Iron(S.I) and TIBC. Results: The most frequent presenting complaints was pallor, incidence of malnourishment was 45.2%,the mean haemoglobin at time of presentation was 6.8± 2.9 g/dl, the mean S.F(ng/ml) and S.I(mcg/dl) at commencement of study was 2059.45 ± 2082.92 and 248.99 ± 92.95 while these values increased to 2610.57 ± 2245.64 and 349.05 ± 110 respectively after 1 year follow-up. All patients received chelation therapy either in form of Deferasirox (mean S.F=1639 ± 962 mean S.I=274.5 ± 76.7) or a combination of Deferasirox and Deferiprone (mean S.F= 5350 ± 3349.7 mean S.I=397.9 ± 128.2). Conclusion: Majority of participants are inadequately transfused and chelation therapy was not effective in preventing iron overload which mandates a need of better and more effective chelators, revision of transfusion guidelines as well as proper counselling regarding attitude and behaviour of caregivers.