|Year : 2019 | Volume
| Issue : 3 | Page : 84-87
Prevalence of glucose-6-phosphate dehydrogenase deficiency and sickle-cell trait among blood donors: Revisited after 10 years in the same institute
Mansour S Aljabry1, Abdulaziz Alhoshan1, Fahad Alrawaf1, Abdullah Alhowidi1, Abdullah Alsahli1, Saad Alrubaia1, Abdulmajeed Alshaikh1, Adnan Alkhayat2, Mohammed Khalid Alabdulaali2
1 Department of Pathology, King Khalid University Hospital, King Saud University, Riyadh, Saudi Arabia
2 Minstry of Health, Kingdom of Saudi Arabia
|Date of Web Publication||14-Nov-2019|
Mansour S Aljabry
Department of Pathology, King Khalid University Hospital, King Saud University, Riyadh
Source of Support: None, Conflict of Interest: None
BACKGROUND: Sickle cell anemia and glucose-6-phosphate dehydrogenase deficiency (G6PD) are the most common causes of inherited hemolytic anemia in Saudi Arabia. Due to high prevalence, some blood transfusion services perform routine blood donor screening for hemoglobin S (HbS) and G6PD enzyme assay. This study was conducted to reassess the prevalence of these disorders after 10 years of the last published report from the same institute. In addition, the utility of donor screening for sickle trait status was also reviewed.
DESIGN: This cross-sectional study has utilized archived data of all blood donors between April 2016 and June 2016. All blood donors who fulfilled the inclusion criteria for blood donation during the study period were enrolled in this study. They all answered a standardized medical questionnaire and have been clinically examined by the blood bank medical staff as well.
MATERIALS AND METHODS: All donors' samples were tested for sickle-cell trait (SCT) by HbS solubility test and capillary electrophoresis and for G6PD enzyme activity by fiuorescent spot test. All investigations were completed within 24 h after blood collection and processed based on the standardized procedures which have been fully accredited by the College of American Pathologists.
RESULTS: Of the 2748 blood donors' samples, there were 2701 (98.3%) males and 47 (1.7%) females. SCT was present in 41 (1.49%), G6PD was detected in 80 (2.91%), and 14 (0.50%) donors were positive for both conditions. All the donors with positive results for both conditions were males.
CONCLUSION: The utility of donor screening for SCT in the central region of KSA is questionable due to the low prevalence of SCT carrier among blood donors and increase of public awareness about HbS carrier status due to wide implementation of premarital screening program for sickle cell and thalassemia. In the same manner, screening of G6PD deficiency should be restricted only to high-risk recipient such as premature neonates.
Keywords: Blood donors, glucose-6-phosphate dehydrogenase deficiency, prevalence, sickle-cell trait, sickle cell anemia
|How to cite this article:|
Aljabry MS, Alhoshan A, Alrawaf F, Alhowidi A, Alsahli A, Alrubaia S, Alshaikh A, Alkhayat A, Alabdulaali MK. Prevalence of glucose-6-phosphate dehydrogenase deficiency and sickle-cell trait among blood donors: Revisited after 10 years in the same institute. J Appl Hematol 2019;10:84-7
|How to cite this URL:|
Aljabry MS, Alhoshan A, Alrawaf F, Alhowidi A, Alsahli A, Alrubaia S, Alshaikh A, Alkhayat A, Alabdulaali MK. Prevalence of glucose-6-phosphate dehydrogenase deficiency and sickle-cell trait among blood donors: Revisited after 10 years in the same institute. J Appl Hematol [serial online] 2019 [cited 2020 Jul 13];10:84-7. Available from: http://www.jahjournal.org/text.asp?2019/10/3/84/271021
| Introduction|| |
Glucose-6-phosphate dehydrogenase deficiency (G6PD) is an X-linked red blood cell (RBC) enzyme deficiency associated with episodes of intravascular hemolysis due to lack of antioxidant function. Hemoglobin S (HbS) is a β-chain variant caused by a single-point mutation, with an increased tendency of abnormal hemoglobin to polymerize at low oxygen tension, resulting in chronic hemolysis and microvascular occlusion.
Sickle cell anemia (SCA) and G6PD are the most common causes of inherited hemolytic anemia in Saudi Arabia,, and their prevalence exhibits regional variation.,,
According to the national premarital screening program in the Kingdom of Saudi Arabia, the overall prevalence rates of sickle-cell trait (SCT) and SCA in the adult population are estimated to be 4.2% and 0.26%, respectively. The lowest prevalence of SCT (1.37%) has been reported from the central and north regions, whereas the highest prevalence of SCT (17%) has been reported from the eastern region.
On the contrary, information about the true prevalence of G6PD deficiency among the Saudi population is patchy and varies due to lack of national screening program. Nevertheless, the published data indicate that G6PD deficiency is a relatively common inherited disorder, with a prevalence ranging between 2% and 9%., Because of the high prevalence rates of SCT and G6PD deficiency, some blood transfusion services in Saudi Arabia perform routine blood donor screening for these disorders, whereas the majority of blood banks rely on the clinical history and medical interview during the donation process.
Screening of blood for G6PD deficiency and HbS is strongly recommended in some clinical conditions such as premature neonate transfusion, exchange transfusion for neonates, and hypertransfusion protocols for SCA and thalassemia patients. Hence, blood bank at the King Khalid University Hospital (KKUH) screens all blood donors due to high frequency of these procedures in the hospital. Furthermore, such donor screening provides a good opportunity to study the prevalence of these inherited disorders in our population.
In 2006, Alabdulaali et al. studied the prevalence of G6PD deficiency and SCT among blood donors at KKUH, and they concluded that 2% were diagnosed for SCT, 0.78% for G6PD deficiency, and 0.35% for both conditions.
The aim of this current study was to assess the current prevalence of these disorders for comparison with previously published report from KKUH. In addition, the utility of donor screening program for SCT will also be reviewed as public awareness regarding hemoglobinopathies has increased tremendously due to widespread implementation of the national premarital screening program.
| Materials and Methods|| |
This retrospective cross-sectional study has utilized the archived data from 2748 blood donors at KKUH in Riyadh, during the period between April 2016 and June 2016. All blood donors who fulfilled the inclusion criteria for blood donation during the study period were enrolled in this study. Hemoglobin level was measured using the standard hemoglobin point-of-care testing (HemoCue® Hb 201+ System). All blood donors must have a hemoglobin level of at least 12.5 g/dl.
They all answered the standardized medical questionnaire that ensures obtaining the relevant information about the donor's health and risk factors that may jeopardize the recipient health. They all have been interviewed and examined by the blood bank medical staff as well.
Blood samples from donors were collected in ethylenediaminetetraacetic acid tubes and kept at 2°C–6°C. Complete blood count (CBC) was performed using Beckman Coulter automated analyzer. Screening for HbS was performed by both solubility test and capillary electrophoresis (capillary2 Sebia), whereas G6PD deficiency was detected by fiuorescent spot test (Boehringer Mannheim Diagnostica, Germany). Normal control and G6PD-deficient sample were included in each testing batch of donor's specimens. All investigations were completed within 24 h after blood collection and processed based on the standardized procedures which have been fully accredited by the College of American Pathologists. The study was approved by the Institutional Review Board of the College of Medicine, King Saud University, Riyadh, Saudi Arabia.
| Results|| |
Of the 2748 enrolled blood donors, 2701 (98.3%) were male, whereas 47 (1.7%) were female. There were 135 (4.9%) positive blood donors comprising of 41 (1.49%) with SCT, 80 (2.91%) had G6PD, and 14 (0.50%) tested positive for both conditions [Figure 1]a and [Figure 1]b. All donors who tested positive for either condition were males.
|Figure 1: (a) Percentages of negative blood donors versus all positive blood donors for sickle-cell trait, glucose-6-phosphate dehydrogenase deficiency or both conditions. (b) Percentages of sickle-cell trait, glucose-6-phosphate dehydrogenase deficiency, and both conditions among postive blood donors|
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All hematological parameters, including hemoglobin (mean 15.21 g/L; normal range 12.9–17.4 g/L), RBC count (mean, 5.02 × 1012/L; normal range: 3.67–5.56 × 1012/L), mean corpuscular volume (mean, 83.3 fi; range 69–94.2 fi), mean corpuscular hemoglobin (mean, 29.1 pg; range 24.4–32.5 pg), and red cell distribution width (mean 13.6; range 12.2–14.7), were within the normal acceptable reference ranges.
| Discussion|| |
The findings of the present study revealed that SCT and G6PD deficiency were present in 1.4% and 2.9% blood donors, respectively, whereas positive donors for both conditions represent about 0.5% only. These observations were consistent with the previously published reports among the blood bank donors in the central region of Saudi Arabia.,
The current study showed a lower prevalence of SCT as compared to the previous study, which was conducted at the same institute (KKUH blood bank) in 2006. This finding could be partially explained by increased public awareness about HbS carrier status among the adult population due to implementation of the national premarital screening program in Saudi Arabia, particularly in the low prevalence areas. Hence, the known SCT carriers do not usually present to blood bank donation as they realized that they will be deferred permanently. Moreover, those carriers can be easily identified and excluded during predonation medical interview. National premarital screening program was stipulated and implemented by a royal decree in February 2004 as a mandatory step before any marriage proposal. As a consequence, all couples have to be tested for thalassemia and SCD using CBC, hemoglobin electrophoresis, iron profile, and sickling test. An appropriate genetic counseling has to be provided for all couples before issuing of any marriage certificate to reduce the prevalence of SCD and thalassemia.
At the short term, the most notable benefits of such program come mainly from the good level of public awareness regarding the inheritance of SCD and thalassemia.
On the other hand, the prevalence of G6PD deficiency in the current study is higher than the previous study due to continuous interregional movement of the population toward the capital of Saudi Arabia and the largest city in the area, which primarily includes the adult men at work age moving from provinces with a high prevalence of G6PD deficiency such as the eastern region.
The fiuorescent spot test is sensitive and reliable for the detection of heterozygous G6PD deficiency in males and homozygous G6PD deficient females, despite some difficulties which might be encountered in the detection of heterozygous female donors. However, female donors in our sample represent few minority (1.7%). Furthermore, the level of G6PD enzyme can be falsely high during hemolysis crisis because reticulocytes contain higher G6PD compared to mature RBC. According to Renzaho et al., almost all published studies about blood transfusion of neonates recommended a routine blood screening for G6PD deficiency to prevent the deleterious effect of raised serum bilirubin in neonates because of their immature liver function. Transfusion of G6PD-deficient RBCs in adult patients did not reveal significant hemolysis or other related adverse outcomes except in some case reports which describedin vivo hemolysis after transfusion of G6PD-deficient blood., However, no solid conclusion can be drawn from such limited data.
| Conclusion|| |
The utility of donor screening for SCT is questionable in the central region of Saudi Arabia due to the low prevalence of SCT carrier among blood donors and increased public awareness about HbS carrier status due to wide implementation of premarital screening program for sickle cell and thalassemia. However, such kind of donor screening is of clinical significance in the eastern and southwestern regions due to the high prevalence of SCT. In the same manner, screening of G6PD deficiency should be restricted only to high-risk recipient such as premature neonates.
Financial support and sponsorship
Confiicts of interest
There are no confiicts of interest.
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