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 Table of Contents  
ORIGINAL ARTICLE
Year : 2019  |  Volume : 10  |  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


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 Publication14-Nov-2019

Correspondence Address:
Mansour S Aljabry
Department of Pathology, King Khalid University Hospital, King Saud University, Riyadh
Saudi Arabia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/joah.joah_22_19

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  Abstract 


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 2019 Dec 13];10:84-7. Available from: http://www.jahjournal.org/text.asp?2019/10/3/84/271021




  Introduction Top


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.[1] 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.[2]

Sickle cell anemia (SCA) and G6PD are the most common causes of inherited hemolytic anemia in Saudi Arabia,[3],[4] and their prevalence exhibits regional variation.[5],[6],[7]

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.[8]

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%.[4],[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.[10]

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.[11] 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.[12]

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 Top


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 Top


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 Top


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.[1],[6]

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.[12] 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.[6]

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.[13]

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.[14]

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.[15] 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.[16] 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.[17] 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.[18],[19] However, no solid conclusion can be drawn from such limited data.


  Conclusion Top


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

Nil.

Confiicts of interest

There are no confiicts of interest.



 
  References Top

1.
Youngster I, Arcavi L, Schechmaster R, Akayzen Y, Popliski H, Shimonov J, et al. Medications and glucose-6-phosphate dehydrogenase deficiency: An evidence-based review. Drug Saf 2010;33:713-26.  Back to cited text no. 1
    
2.
Manwani D, Frenette PS. Vaso-occlusion in sickle cell disease: Pathophysiology and novel targeted therapies. Blood 2013;122:3892-8.  Back to cited text no. 2
    
3.
Al-Qurashi MM, El-Mouzan MI, Al-Herbish AS, Al-Salloum AA, Al-Omar AA. The prevalence of sickle cell disease in Saudi children and adolescents. A community-based survey. Saudi Med J 2008;29:1480-3.  Back to cited text no. 3
    
4.
Alharbi KK, Khan IA. Prevalence of glucose-6-phosphate dehydrogenase deficiency and the role of the A- variant in a Saudi population. J Int Med Res 2014;42:1161-7.  Back to cited text no. 4
    
5.
Memish ZA, Owaidah TM, Saeedi MY. Marked regional variations in the prevalence of sickle cell disease and β-thalassemia in Saudi Arabia: Findings from the premarital screening and genetic counseling program. J Epidemiol Glob Health 2011;1:61-8.  Back to cited text no. 5
    
6.
Alhamdan NA, Almazrou YY, Alswaidi FM, Choudhry AJ. Premarital screening for thalassemia and sickle cell disease in Saudi Arabia. Genet Med 2007;9:372-7.  Back to cited text no. 6
    
7.
El-Hazmi MA, Warsy AS. Appraisal of sickle-cell and thalassaemia genes in Saudi Arabia. East Mediterr Health J 1999;5:1147-53.  Back to cited text no. 7
    
8.
Memish ZA, Saeedi MY. Six-year outcome of the national premarital screening and genetic counseling program for sickle cell disease and β-thalassemia in Saudi Arabia. Ann Saudi Med 2011;31:229-35.  Back to cited text no. 8
[PUBMED]  [Full text]  
9.
el-Hazmi MA. Clinical and haematological diversity of sickle cell disease in Saudi children. J Trop Pediatr 1992;38:106-12.  Back to cited text no. 9
    
10.
Francis RO, Jhang JS, Pham HP, Hod EA, Zimring JC, Spitalnik SL, et al. Glucose-6-phosphate dehydrogenase deficiency in transfusion medicine: The unknown risks. Vox Sang 2013;105:271-82.  Back to cited text no. 10
    
11.
New HV, Berryman J, Bolton-Maggs PH, Cantwell C, Chalmers EA, Davies T, et al. Guidelines on transfusion for fetuses, neonates and older children. Br J Haematol 2016;175:784-828.  Back to cited text no. 11
    
12.
Alabdulaali MK, Alayed KM, Alshaikh AF, Almashhadani SA. Prevalence of glucose-6-phosphate dehydrogenase deficiency and sickle cell trait among blood donors in Riyadh. Asian J Transfus Sci 2010;4:31-3.  Back to cited text no. 12
[PUBMED]  [Full text]  
13.
Al Sulaiman A, Suliman A, Al Mishari M, Al Sawadi A, Owaidah TM. Knowledge and attitude toward the hemoglobinopathies premarital screening program in Saudi Arabia: Population-based survey. Hemoglobin 2008;32:531-8.  Back to cited text no. 13
    
14.
Warsy AS, El-Hazmi MA. G6PD deficiency, distribution and variants in Saudi Arabia: An overview. Ann Saudi Med 2001;21:174-7.  Back to cited text no. 14
    
15.
Wang FL, Boo NY, Ainoon O, Wong MK. Comparison of detection of glucose-6-phosphate dehydrogenase deficiency using fiuorescent spot test, enzyme assay and molecular method for prediction of severe neonatal hyperbilirubinaemia. Singapore Med J 2009;50:62-7.  Back to cited text no. 15
    
16.
Frank JE. Diagnosis and management of G6PD deficiency. Am Fam Physician 2005;72:1277-82.  Back to cited text no. 16
    
17.
Renzaho AM, Husser E, Polonsky M. Should blood donors be routinely screened for glucose-6-phosphate dehydrogenase deficiency? A systematic review of clinical studies focusing on patients transfused with glucose-6-phosphate dehydrogenase-deficient red cells. Transfus Med Rev 2014;28:7-17.  Back to cited text no. 17
    
18.
Kumar P, Sarkar S, Narang A. Acute intravascular haemolysis following exchange transfusion with G-6-PD deficient blood. Eur J Pediatr 1994;153:98-9.  Back to cited text no. 18
    
19.
Shalev O, Bogomolski-Yahalom V, Sharon R. Hemolysis following transfusion of erythrocytes from a donor with G6PD deficiency and beta-thalassemia minor. Isr J Med Sci 1993;29:214-6.  Back to cited text no. 19
    


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