THE EFFECTS OF ALPHA THALASSAEMIA ON THE HAEMATOLOGICAL AND CLINICAL STATUS OF SICKLE CELL ANAEMIA PATIENTS AT THE NATIONAL HOSPITAL ABUJA, NIGERIA

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THE EFFECTS OF ALPHA THALASSAEMIA ON THE HAEMATOLOGICAL AND CLINICAL STATUS OF SICKLE CELL ANAEMIA PATIENTS AT THE NATIONAL HOSPITAL ABUJA, NIGERIA

Abstract

Background: The severity of Sickle Cell Anaemia severity varies in different individuals and populations. Some individuals have very mild disease while others have severe disease. The co-inheritance of alpha thalassaemia and sickle cell anaemia provides an interesting example of how separate globin gene mutations can influence the clinical manifestations of an abnormal gene. It was therefore important for the specific clinical effects of alpha thalassaemia in sickle cell anaemia to be determined in Abuja, Nigeria.
Aims and Objectives: The aim of the study was to determine the effects of alpha thalassaemia on the haematological and clinical status of patients with sickle cell anaemia at the National Hospital Abuja
Methods: Fifty one Sickle Cell anaemia patients who were recruited from 24th January 2013 to 25th April 2013 were enrolled in a cross-sectional observational study. The diagnosis of both haemoglobinopathies were made using PCR. Demographic, clinical and biochemical data were collected using each patient’s case note. Sickle Cell anaemia Severity score was calculated using the Bayesian Network.
Results and Analysis: The prevalence of alpha thalassaemia among Sickle Cell Anaemia patients at the National Hospital Abuja was found to be 5.9% with a gene frequency of 0.06. Only one type of alpha thalassaemia –α3.7 was found.
There was no statistically significant difference in the Hb concentration, MCV, MCH and MCHC of those with alpha thalassaemia compared with those who had normal alpha globin gene. A high prevalence of priapism (55%) was found in the male population. It affected both those who had alpha thalassaemia and normal alpha globin gene. The study implied that the co-inheritance of alpha thalassaemia does not confer milder disease using the Bayesian Network to calculate Sickle Cell Disease Severity Score.
Conclusion: In this study, Alpha thalassaemia does not influence Sickle Cell anaemia using the Bayesian network. Hence it does not cause mild disease. This might be due to other co-morbid conditions in the patients with alpha thalassaemia that are not related to sickle cell anaemia.

THE EFFECTS OF ALPHA THALASSAEMIA ON THE HAEMATOLOGICAL AND CLINICAL STATUS OF SICKLE CELL ANAEMIA PATIENTS AT THE NATIONAL HOSPITAL ABUJA, NIGERIA

 

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