BACKGROUND: Sickle cell anaemia is prevalent in sub Saharan Africa. While α+-thalassaemia is known to modulate sickle cell anaemia, its magnitude and significance in Uganda have hitherto not been described. OBJECTIVES: To determine the prevalence of α+thalassaemia among sickle cell anaemia patients in Mulago Hospital and to describe the clinical and laboratory findings in these patients. METHODS: A cross sectional study was carried out on patients with sickle cell anaemia in Kampala. Dried blood spots were used to analyze for the deletional α+ thalassaemia using multiplex polymerase chain reaction. RESULTS: Of the 142 patients with sickle cell anaemia, 110 (77.5%) had the αα+thalassaemia deletion. The gene frequency of (-α) was 0.425. Ninety one percent (100/110) of those with α+thalassaemia were heterozygous (αα/α-). Amongst the patients older than 60 months, 15 (83.3%) of those without αα+thalassaemia had significant hepatomegaly of greater than 4 cm compared to 36 (45.6%) of those with α+thalassaemia (p=0.003). CONCLUSION: The gene frequency of (-α) of 0.425 noted in this study is higher than that reported from many places in Africa. Concurrent alpha thalassemia might be a protective trait against significant hepatomegaly in sickle cell anaemia patients more than 60 months of age at Mulago hospital.
BACKGROUND:Sickle cell anaemia is prevalent in sub Saharan Africa. While α+-thalassaemia is known to modulate sickle cell anaemia, its magnitude and significance in Uganda have hitherto not been described. OBJECTIVES: To determine the prevalence of α+thalassaemia among sickle cell anaemiapatients in Mulago Hospital and to describe the clinical and laboratory findings in these patients. METHODS: A cross sectional study was carried out on patients with sickle cell anaemia in Kampala. Dried blood spots were used to analyze for the deletional α+ thalassaemia using multiplex polymerase chain reaction. RESULTS: Of the 142 patients with sickle cell anaemia, 110 (77.5%) had the αα+thalassaemia deletion. The gene frequency of (-α) was 0.425. Ninety one percent (100/110) of those with α+thalassaemia were heterozygous (αα/α-). Amongst the patients older than 60 months, 15 (83.3%) of those without αα+thalassaemia had significant hepatomegaly of greater than 4 cm compared to 36 (45.6%) of those with α+thalassaemia (p=0.003). CONCLUSION: The gene frequency of (-α) of 0.425 noted in this study is higher than that reported from many places in Africa. Concurrent alpha thalassemia might be a protective trait against significant hepatomegaly in sickle cell anaemiapatients more than 60 months of age at Mulago hospital.
Authors: A E Felice; B Webber; A Miller; S M Mayson; H F Harris; J B Henson; M E Gravely; T H Huisman Journal: Am J Hematol Date: 1979 Impact factor: 10.047
Authors: D R Higgs; B E Aldridge; J Lamb; J B Clegg; D J Weatherall; R J Hayes; Y Grandison; Y Lowrie; K P Mason; B E Serjeant; G R Serjeant Journal: N Engl J Med Date: 1982-06-17 Impact factor: 91.245
Authors: Oladele S Olatunya; Dulcineia M Albuquerque; Adekunle Adekile; Fernando F Costa Journal: J Clin Lab Anal Date: 2018-08-20 Impact factor: 2.352
Authors: Aimé Lukusa Kazadi; René Makuala Ngiyulu; Jean Lambert Gini-Ehungu; Jean Marie Mbuyi-Muamba; Michel Ntetani Aloni Journal: Anemia Date: 2017-01-30