Effects of Hydroxyurea Treatment on Haematological Parameters and Neurological Functions in Patients of Sickle Beta Thalassemia
Journal Title: Journal of Medical Science And clinical Research - Year 2017, Vol 5, Issue 7
Abstract
Background: Sickle beta thalassemia (HbS-β-Thal) is a disorder which represents the double heterozygous state for the Sickle cell anaemia and the beta - thalassemia genes. It constitutes one of the major genetic haematological disorders in Odisha. The aim of the study is to determine the haematological profile before and after hydroxyurea treatment and its effects on neurological function. Methods: Blood samples are collected from 45 diagnosed cases of sickle beta thalassemia after taking informed consent as well as due ethical Committee approval. Screening is done by Sickling test and Haemoglobin variants are analysed by fully automated capillary zone electrophoresis. Hydroxyurea is given in appropriate doses and its effect on haematological parameters and neurological functions are studied. Results: In our observation the fetal hemoglobin is raised to 30.63% ranges from 27% to 36.7%. Adult haemoglobin (HbA> 3.5%) and Sickle cell haemoglobin (HbS > 67%) was taken as determinant for Sickle cell beta thalassemia. We observed high percentage of HbS ranging from 45.9% to 82.3% and the mean is being 69.45%. There are overall increases in HbF by 6% with reductions in the frequency of blood transfusion and no neurological deficit observed after hydroxyurea treatment. Conclusion: In sickle beta thalassemia, there are overall increases in HbF with reductions in the frequency of blood transfusion after hydroxyurea treatment. Moreover molecular diagnosis is required for β-Globin Gene mutations. Abbreviations: HbS-β-Thal -Sickle beta thalassemia, Hb A- Adult haemoglobin, HbS- sickle cell haemoglobin, HbF-foetal haemoglobin
Authors and Affiliations
Dharma Niranjan Mishra
Keywords
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