Asian Hematology Research Journal

Exploring the Coexistence of Glucose-6-Phosphate Dehydrogenase Deficiency in Sickle Cell Anaemia Patients: Insights from Maiduguri, Nigeria's North-East Region

Kawu YA *

Department Haematology, State Specialist Hospital Maiduguri, Borno State, Nigeria.

Farouk AG *

Department of Paediatrics, Faculty of Clinical Sciences, College of Medical Sciences, University of Maiduguri, Borno State, Nigeria.

Kunduli Y *

Department Haematology, State Specialist Hospital Maiduguri, Borno State, Nigeria.

Talba HA *

Department Haematology, State Specialist Hospital Maiduguri, Borno State, Nigeria.

Kukawa YM *

Department Haematology, State Specialist Hospital Maiduguri, Borno State, Nigeria.

Gubio BG *

Department of Chemical Pathology, University of Maiduguri Teaching Hospital, Nigeria.

*Author to whom correspondence should be addressed.

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Abstract

Background: Sickle cell anaemia (SCA) and Glucose-6-phosphate dehydrogenase (G-6-PD) deficiency are inherited red cell disorders associated with chronic haemolysis that have a similar pattern of occurrence in malaria-endemic areas, including Nigeria. 

Objectives: This study aims to determine the effect of co-inheritance of red blood cell G-6-PD deficiency and SCA regarding some clinical parameters.

Methods: This cross-sectional study was conducted over 13 months involving 235 SCA  patients and 235 voluntary HbAA controls. Patients were HbSS as confirmed by Hb electrophoresis in an alkaline medium (pH 8.4-8.6) in a steady state. Quantitative G-6-PD enzyme activity among the study population was assayed using the spectrophotometric method.

Results: The prevalence of G-6-PD deficiency was similar in patients (29.3%) and controls (25.5%). Only 3.8% of patients had total G-6-PD deficiency, and 25.5% had partial deficiency. The mean (SD) G-6-PD activity of patients was totally deficient; 1.49(0.43), partially deficient; 4.95(1.45), and normal; 10.39(2.66). Similarly, G-6-PD activity in controls was totally deficient; 1.62(0.36), partially deficient; 4.93(1.54) and normal 9.00(1.89). The mean age at first transfusion (±SD) was lower in patients with total G-6-PD deficiency (4.89 years ± 3.96) when compared with patients with normal G-6-PD activity (10.73 years ± 2.27).

Conclusion: The prevalence of G-6-PD deficiency is high in both SCA patients and normal controls. Sickle cell anaemia patients with co-existing G-6-PD deficiency commence transfusion at a younger age than those without G-6-PD deficiency.

Keywords: Co-existing, deficiency, disorder, G-6-PD, sickle cell, hereditary

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