Fasola Foluke Atinuke, Fowodu Florence Olamide, Shokunbi Wuraola Adebola, Kotila Taiwo Rachel
Department of Haematology, College of Medicine, University of Ibadan, Ibadan, Nigeria.
Department of Haematology, University College Hospital, Ibadan, Nigeria.
Niger Postgrad Med J. 2019 Apr-Jun;26(2):118-122. doi: 10.4103/npmj.npmj_29_19.
Sickle cell disease (SCD) and glucose-6-phosphate dehydrogenase (G6PD) deficiency are inherited disorders associated with chronic haemolysis. Therefore, coinheritance of both disorders could worsen haemolysis in the former and compound a haemolytic crisis. This study compared clinical and laboratory features of deficient and non-deficient SCD patients and the G6PD activities of SCD patients and apparently healthy controls.
This is a case-control study of 175 SCD patients and 166 non-SCD controls. G6PD assay was carried out on haemolysate from washed red cells. The G6PD activity was measured by spectrophotometry.
The mean age of patients and controls was 27.3 ± 9.4 and 35.9 ± 9.7 years, respectively, with 75 (46.2%) and 87 (52.4%) being males, respectively. G6PD activity was similar in cases and controls (6.7 ± 3.3 vs. 6.9 ± 3.0 IU/gHb), respectively (P = 0.6). The prevalence of G6PD deficiency was higher in patients than controls (28.6% vs. 22.3%, P = 0.18), and SCD patients were twice more likely to have enzyme activities below 3.0 IU/gHb. No significant difference was observed in the clinical parameters between deficient and non-deficient patients. Deficient patients were more likely to have lower haematocrit (22.8 ± 3.9% vs. 24.5 ± 5%, P = 0.04) and non-significantly higher bilirubin and reticulocyte counts. Furthermore, in patients, severe deficiency resulted in higher bilirubin than in those with mild deficiency (60.5 vs. 21.7 IU/L, P < 0.001). G6PD activity correlated positively with haematocrit (r = 0.91, P = 0.01) and mean corpuscular haemoglobin concentration (r = 0.17, P = 0.02).
Coinheritance of both disorders could worsen haemolysis in SCD patients, and care should, therefore, be taken in the choice of drugs in deficient SCD patients.
镰状细胞病（SCD）和葡萄糖-6-磷酸脱氢酶（G6PD）缺乏症是与慢性溶血相关的遗传性疾病。因此，这两种疾病的共同遗传可能会使前者的溶血情况恶化，并引发溶血危象。本研究比较了G6PD缺乏和不缺乏的SCD患者的临床和实验室特征，以及SCD患者和明显健康对照者的G6PD活性。
这是一项针对175例SCD患者和166例非SCD对照者的病例对照研究。对洗涤红细胞的溶血产物进行G6PD检测。通过分光光度法测量G6PD活性。
患者和对照者的平均年龄分别为27.3±9.4岁和35.9±9.7岁，男性分别为75例（46.2%）和87例（52.4%）。病例组和对照组的G6PD活性相似（分别为6.7±3.3和6.9±3.（IU/gHb），P = 0.6）。患者中G6PD缺乏症的患病率高于对照组（28.6%对22.3%，P = 0.18），SCD患者酶活性低于3.0 IU/gHb的可能性是对照组的两倍。G6PD缺乏和不缺乏的患者在临床参数上未观察到显著差异。G6PD缺乏的患者更可能有较低的血细胞比容（22.8±3.9%对24.5±5%，P = 0.04），胆红素和网织红细胞计数略高但无统计学意义。此外，在患者中，严重缺乏者的胆红素水平高于轻度缺乏者（60.5对21.7 IU/L，P < 0.001）。G6PD活性与血细胞比容呈正相关（r = 0.91，P = 0.01），与平均红细胞血红蛋白浓度呈正相关（r = 0.17，P = 0.02）。
这两种疾病的共同遗传可能会使SCD患者溶血情况恶化，因此，对于G(6)PD缺乏的SCD患者，用药时应谨慎。
