INHERITED HAEMOGLOBIN DISORDERS AND THEIR CORRELATION WITH COMPLETE BLOOD COUNT AND PERIPHERAL BLOOD SMEAR. AN ANALYSIS OF 50 CASES.
Pankaj Bahadur Nepali*, Bhawani Manandhar, Sachin Kumar, Raju S., Somendra M.
ABSTRACT
Introduction: Inherited hemoglobin disorders present a significant health problem all over the world. Due to migration, this problem is increasing day by day. No definitive data regarding the frequency of hereditary haemoglobin disorders are available in Nepal. Iron deficiency is most widely prevalent in the country and beta thalassaemia is also common. Objective: The aim of the study is to determine the relative frequency of inherited haemoglobin disorders. Methods: This retrospective cross-sectional study was carried out in the Department of Haematology Mangalam diagnostics center from July 2014 to January 2016. A total of 50 cases were included in this study. Blood samples of specific amount were taken from all these individuals for estimation of CBC, red cell variables and Hb electrophoresis. Results: Hb electrophoresis revealed normal Hb pattern in 76% of cases, followed by (Hb S, Hb A and Hb F), Hbs/β+ Thalassaemia (66.6%), (Hb A Hb A2 and Hb F) Heterozygous db Thalassaemia (16.6%). HPFH and HBAS (Sickle Trait) are only 8.3% cases in each. In this study, a total of 24% abnormal Hb pattern were detected. Conclusion: The finding of abnormal Hb pattern on Hb electrophoresis in this study among the cases indicated the high frequency of inherited haemoglobin disorders among our population and hence warranted detection of these disorders and subsequent appropriate genetic counseling.
Keywords: Inherited haemoglobin disorders, Hb electrophoresis, Genetic counseling.
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