| Category | ORIGINAL_ARTICLE |
|---|---|
| Authors | Dharma Niranjan Mishra, Rabindra Kumar Jena |
| Abstract | Background: Sickle cell beta thalassemia (Hb S/β Thal) is an inherited form of sickle cell disease that affects red blood cells both in the production of abnormal hemoglobin as well as the decreased synthesis of beta globin chains. It constitutes one of the major genetic haematological disorders in Odisha. The aim of the study is to determine the haematological profile of patients suffering from sickle beta thalassemia. Methods: After obtaining informed consent and ethical committee approval, blood samples were collected from 45 diagnosed cases of sickle beta thalassemia. Screening was done by Sickling test and haemoglobin variants were analysed by fully automated capillary zone electrophoresis. Results: In our observation, the fetal hemoglobin (HbF) was raised to 21.63% (SD± 5.24) ranging from 11% to 32.7% and adult haemoglobin (Hb A2) was also raised with mean value 4.12 (SD±1.00) which is >3.5%, suggestive of sickle cell-β-thalassemia. We observed high percentage of sickle cell haemoglobin (HbS) ranging from 45.9% to 82.3%, the mean was being 69.45% (SD±4.35). Conclusion: The present study highlights the co-inheritance of β-thalassemia and sickle cell gene. Molecular diagnosis is required for β-Globin Gene mutations in this state. KEYWORDS: Anaemia, haemoglobin variants, sickle cell beta thalassemia, β-Globin Gene |
| Year | 2017 |
| Month | October |
| Volume | 6 |
| Issue | 4 |
| Published On | 28 Oct 2017 |
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