EVALUATION OF GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY IN PATIENTS WITH SICKLE CELL ANEMIA

Abstract

Objective: The aim of this study was to evaluate patients with a diagnosis of Sickle Cell Anemia (SCA) for Glucose-6-Phosphate Dehydrogenase (G6PD) enzyme deficiency. Methodology: In our study, patients diagnosed with SCA who presented to the Pediatric Hematology and Oncology Clinic at the Adana Faculty of Medicine, Health Sciences University, Adana City Training and Research Hospital, between August 1, 2022, and August 1, 2023, were evaluated. G6PD enzyme data from routine tests performed for the patients were recorded from the patient files or the hospital system. Results: A total of 23 patients diagnosed with Sickle Cell Anemia (SCA) were included in the study. 65.2% (n=15) of the patients were female, and 34.8% (n=8) were male. The ages of the cases ranged from 4 to 30 years, with a median age of 12. Among the cases, 20 were within the age range of 0-18 years (87%), while 3 cases (13%) were over 18 years old. The median G6PD value was found to be 26.28 U/g Hb (2.22-36.98). G6PD deficiency was detected in 2 patients (8.7%), while it was not detected in 21 patient Conclusion: Screening for G6PD deficiency is necessary in patients with Sickle Cell Anemia (SCA) to prevent deterioration of their condition during treatment. The co-inheritance of both diseases can worsen hemolysis in SCA patients. Therefore, caution should be exercised in drug selection for SCA patients with G6PD enzyme deficiency.