Haemoglobin Patterns in Patients with Sickle Cell Haemoglobinopathies

Eman A. Ajjack¹, Hiba A. Awooda² and Sana Eltahir Abdalla^3,

¹Department of Hematology, Omdurman Islamic University, Faculty of Medical Laboratories, Omdurman Sudan

²Department of Physiology, Al Neelain University, Faculty of Medicine, Khartoum Sudan

³Al Neelain Medical Research Centre, Al Neelain University, Faculty of Medicine, Khartoum Sudan

Cite this paper:
Eman A. Ajjack, Hiba A. Awooda and Sana Eltahir Abdalla. Haemoglobin Patterns in Patients with Sickle Cell Haemoglobinopathies. International Journal of Hematological Disorders. 2014; 1(1):8-11. doi: 10.12691/ijhd-1-1-2

Abstract

Background: Hemoglobinopathy is a group of inherited disorders characterized by structural variations of the hemoglobin molecule; and sickle cell disease constitutes one of the major genetic blood disorders in Sudan. The aim of this study was to determine the haemoglobin patterns of patients with sickle cell haemoglobinopathies. Methods: Blood samples were collected from 70 subjects diagnosed or suspected to have sickle cell disease. Blood samples were also taken from 30 control patients. Screening was done by sickling test and capillary electrophoresis was done. Results: 37 patients (52.9%) showed AS, 1 patient (1.4%) showed AS/C, 8 patients (11.4%) showed S/ßThalassaemia, 1 patient (1.4%) showed S/C, 3 patients (4.3%) showed S/D and 20 patients (28.6%) showed SS. The mean level of Hb showed lower level in patients group while HbA2 showed no significant change and HbF and HbS showed different levels according to the type of haemoglobinopathy. Conclusion: Different variants of sickle cell haemoglobinopathies were identified; AS, AS/C, S/ßThalassaemia, S/C, S/D and SS patterns were reported. Haemoglobin A2 have no significant difference in patients with sickle cell disease, while Hb F and Hb S show significant elevation.

Keywords:
Hemoglobinopathy sickle cell disease haemoglobin patterns

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References:

[1]	Wilson M, Forsyth P, Whiteside J: Haemoglobinopathy and sickle cell disease. Continuing Education in Anaesthesia, Critical Care & Pain 2009, 10.
[2]	Ohls RK CR: Development of Haematopoeitic system. In: Nelson Textbook of Paediatrics. Edited by Jenson HB e, 17th edn; 2004.
[3]	Haima GK: Haemoglobinopathy. In: The American Heritage Medical Dictionary. vol. 8th: Houghton Mifflin Company; 2009.
[4]	Herrick J: Peculiar elongated and sickle-shaped red blood corpuscles in a case of severe anemia. Arch Intern Med 1910, 6: 517-521.
[5]	Firth PG HC: Sickle cell disease and anesthesia. Anesthesiology 2004, 101: 766-785.
[6]	Serjeant GR, Serjeant BE: Sickle Cell Disease, 3rd edn: Oxford: Oxford University Press.; 2001.
[7]	Weatherall D, Akinyanju O, Fucharoen S, Olivieri N, Musgrove P: Disease Control Priorities in Developing Countries. In. Edited by Jamison DT ea: Oxford University Press; 2006: 663-680.
[8]	Taiwo IA, Oloyede OA, Dosumu AO: Frequency of sickle cell genotype among the Yorubas in Lagos: Implications for the level of awareness and genetic counseling for sickle cell disease in Nigeria. J Community Genet 2011, 2: 13-18.
[9]	Nnaji GA, Ezeagwuna DA, Nnaji I, Osakwe JO, Nwigwe AC, Onwurah OW: Prevalence and pattern of sickle cell disease in premarital couples in Southeastern Nigeria. Niger J Clin Pract 2013, 16(3): 309-314.
[10]	World Medical Association Medical Ethics Committee,Updating the WMA Declaration of Helsinki. Wld Med J 1999, 45: 11-13.
[11]	Hedlund B: Haemoglobinopathies and thalassaemias. In: Haemoglobins of human embryos, fetuses, and neonates. Edited by Fairbanks V. New York; 1980.
[12]	Tarer V, Etienne-Julan M, Diara JP, Belloy MS, Mukizi-Mukaza M, Elion J, Romana M: Sickle cell anemia in Guadeloupean children: pattern and prevalence of acute clinical events. Eur J Haematol 2006, 76(3): 193-199.
[13]	Al-Allawi NA, Al-Dousky AA: Frequency of haemoglobinopathies at premarital health screening in Dohuk, Iraq: implications for a regional prevention programme. East Mediterr Health J 2010, 16(4): 381-385.
[14]	Bayoumi RA, Abu Zeid YA, Abdul Sadig A, Awad Elkarim O: Sickle cell disease in Sudan. Trans R Soc Trop Med Hyg 1988, 82(1): 164-168.
[15]	Altay C, Yilgor E, Beksac S, Gurgey A: Premarital screening of hemoglobinopathies: a pilot study in Turkey. Hum Hered 1996, 46(2): 112-114.
[16]	RK Ohls, Christensen R: Development of Haematopoeitic system. In: Nelson Textbook of Paediatrics. Edited by RE Behram, RM Kliegman, Jenson H, 17th edn: Philadelphia: Saunders; 2004: 1601-1604.
[17]	Shirley L: Haemoglobinopathies and Thalassaemias. In: The ABCs of Lab Evaluation. vol. 35; 2009.
[18]	Cotton F, Lin C, Fontaine B, Gulbis B, Janssens J, Vertongen F: Evaluation of a capillary electrophoresis method for routine determination of hemoglobins A2 and F. Clin Chem 1999, 45(2): 237-243.
[19]	Mohamed AO: Sickle cell disease in the Sudan. Clinical and biochemical aspects. Minireview based on a doctoral thesis. Ups J Med Sci 1992, 97(3): 201-228.
[20]	Molineaux L, Fleming AF, Cornille-Brogger R, Kagan I, Storey J: Abnormal haemoglobins in the Sudan savanna of Nigeria. III. Malaria, immunoglobulins and antimalarial antibodies in sickle cell disease. Ann Trop Med Parasitol 1979, 73(4): 301-310.
[21]	Memish ZA, Owaidah TM, Saeedi MY: Marked regional variations in the prevalence of sickle cell disease and beta-thalassaemia in Saudi Arabia: findings from the premarital screening and genetic counseling program. J Epidemiol Glob Health 2011, 1(1): 61-68.
[22]	Alhamdan NA, Almazrou YY, Alswaidi FM, Choudhry AJ: Premarital screening for thalassaemia and sickle cell disease in Saudi Arabia. Genet Med 2007, 9(6): 372-377.
[23]	Al-Mendalawi MD: RE: Six-year outcome of the national premarital screening and genetic counseling program for sickle cell disease and beta-thalassaemia in Saudi Arabia. Ann Saudi Med, 31(6): 661.