Sickle cell-haemoglobin E (HbSE) compound heterozygosity: A clinical and haematological study

International Journal of Laboratory Hematology

Volumn 29, Issue 4, 2007, Pages 292-301

  Knox Macaulay, H H M ^a,d   Ahmed, M M ^b   Gravell, D ^c   Al Kindi, S ^a   Ganesh, A ^c  

^a SULTAN QABOOS UNIVERSITY   (Oman)

^b NIZWA HOSPITAL   (Oman)

^c SULTAN QABOOS UNIVERSITY HOSPITAL   (Oman)

^d NONE   (United Kingdom)

Author keywords

Compound heterozygotes; Haemoglobinopathy; SE; Sickling

Indexed keywords

ALPHA GLOBIN; BETA GLOBIN; HEMOGLOBIN S;

ACUTE CHEST SYNDROME; ADULT; AGED; ALPHA THALASSEMIA; ARTICLE; BLOOD EXAMINATION; BONE PAIN; CELL STRUCTURE; CHILD; CHROMATOGRAPHY; CONTROLLED STUDY; ERYTHROCYTE; FEMALE; FRONTAL BOSSING; HEMATOLOGY; HEMOGLOBIN DETERMINATION; HEMOGLOBINOPATHY; HEMOLYSIS; HETEROZYGOSITY; HOSPITAL; HUMAN; HUMAN CELL; MAJOR CLINICAL STUDY; MALE; MEAN CORPUSCULAR HEMOGLOBIN; MEDICAL LITERATURE; OMAN; PERIPHERAL OCCLUSIVE ARTERY DISEASE; PREVALENCE; PRIORITY JOURNAL; RECURRENT INFECTION; SICKLE CELL; URINARY TRACT INFECTION;

ANEMIA, SICKLE CELL; FEMALE; HEMOGLOBIN, SICKLE; HETEROZYGOTE; HUMANS; MALE; OMAN; PEDIGREE; RETROSPECTIVE STUDIES;

EID: 34447269181     PISSN: 17515521     EISSN: 1751553X     Source Type: Journal    
DOI: 10.1111/j.1365-2257.2006.00886.x     Document Type: Article

References (30)

1. Aksoy M. (1960) The hemoglobin E syndromes. II. Sickle cell-hemoglobin E disease. Blood 15, 610 613.
2. Aksoy M. Lehmann H. (1957) The first observation of sickle-cell haemoglobin E disease. Nature 179, 1248 1249.
3. Al-Riyami A. Ebrahim G.J. (2003) Genetic blood disorders survey in the Sultanate of Oman. Journal of Tropical Pediatrics 49 (Suppl. 1 i1 i20.
4. Allison A.C. (1964) Polymorphism and natural selection in human populations. Cold Spring Harbor Symposia on Quantitative Biology 29, 137 149.
5. Almeida A.M., Henthorn J.S. Davies S.C. (2001) Neonatal screening for haemoglobinopathies: the results of a 10-year programme in an English Health Region. British Journal of Haematology 112, 32 35.
6. Altay C., Niazi G.A. Huisman T.H.J. (1976-1977) The combination of HbS and HbE in a black female. Hemoglobin 1, 103 110.
7. Beutler E., Blume K.G., Kaplan J.C., Lohr G.W., Ramot B. Valentine W.N. (1979) International Committee for Standardization in Haematology. Recommended screening test for glucose-6-phophate dehydrogenase (G-6-PD) deficiency. British Journal of Haematology 43, 465 467.
8. Chotivanich K., Udomsangpetch R., Pattanapanyasat K., Chierakul W., Simpson J., Looareesuwan S. White N. (2002) Hemoglobin E: a balanced polymorphism protective against high parasitemias and thus severe P falciparum malaria. Blood 100, 1172 1176.
9. Daar S.F. (2000) Haemoglobinopathies in the Sultanate of Oman: A Study of Clinically Significant Beta Globin Gene Mutations. MD Thesis. pp. 181 188. University of Bristol, UK.
10. Eichhorn R.F., Buurke E.J., Blok P., Berends M.J.H. Jansen C.L. (1999) Sickle cell-like crisis and bone marrow necrosis associated with parvovirus B19 infection and heterozygosity for haemoglobins S and E. Journal of Internal Medicine 245, 103 106.
11. Engelstad B.L. (1982) Functional asplenia in haemoglobin SE disease. Clinical Nuclear Medicine 7, 100 102.
12. Fairbanks V.F., Gilchrist G.S., Brimhall B., Jereb J.A. Goldston E.C. (1979) Hemoglobin E trait re-examined: a cause of microcytosis and erythocytosis. Blood 53, 109 115.
13. Flint J., Harding R.M., Boyce A.J. Clegg J.B. (1998) The population genetics of the haemoglobinopathies. Clinical Haematology (Sickle Cell Disease and Thalassaemia) 11, 1 51.
14. Friedman M.J. (1979) Oxidant damage mediates variant red cell resistance to malaria. Nature 280, 245 247.
15. Gibbons R., Higgs D.R., Olivieri N.F. Wood W.G. (2001) The β and δβ thalassaemias in association with structural haemoglobin variants; part 3 - clinical features of the thalassaemias - haemoglobin Eβ thalassaemia. In : Thalassaemia Syndromes (ed. D.J. Weatherall J.B. Clegg pp. 421 439. Blackwell Science Ltd, Oxford, UK.
16. Gupta R., Jarvis M. Yardumian A. (2000) Compound heterozygosity for haemoglobin S and haemoglobin E. British Journal of Haematology 108, 463.
17. Hardy M.J. Ragbeer M.S. (1985) Homozygous HbE and HbSE disease in a Saudi family. Hemoglobin 9, 47 52.
18. Huisman T.H.J., Carver M.F.H. Efremov G.D. (1996) Hb Muscat, Hb Dhofar, HbS Oman. In : A Syllabus of Human Haemoglobin Variants (ed T.H.J. Huisman pp. 170, 192, 354. The Sickle Cell Anemia Foundation, Augusta, Georgia, USA.
19. Hutagalung R., Wilairatana P., Looareesuwan S., Brittenham G.M., Aikawa M. Gordeuk V.R. (1999) Influence of haemoglobin E trait on the severity of falciparum malaria. Journal of Infectious Diseases 179, 283 286.
20. Lewis S.M. Roper D. (2001) Laboratory methods used in the investigation of the haemolytic anaemias. In : Dacie and Lewis Practical Haematology (ed. S.M. Lewis, B.J. Bain I. Bates 9th edn. pp. 149 166. Churchill Livingstone, London.
21. Lorey F.W., Arnopp J. Cunningham G.C. (1996) Distribution of hemoglobinopathy variants by ethnicity in a multiethnic state. Genetics Epidemiology 13, 501 512.
22. Platt O.S. Nathan D.G. (1987) Sickle cell disease. In : Hematology of Infancy and Childhood (ed. D.G. Nathan F.A. Oski pp. 671, 674. W.B. Saunders, Philadelphia, PA.
23. Powars D.R. (1991) Sickle cell anemia: βs-gene cluster haplotypes as prognostic indicators of vital organ failure. Seminars in Hematology 28, 202 208.
24. Ramahi A.J., Lewkow L.M., Dombrowski M.P. Bottoms S.F. (1988) Sickle cell E hemoglobinopathy and pregnancy. Obstetrics & Gynecology 71, 493 495.
25. Rey K.S., Unger C.A., Rao S.P. Miller S.T. (1991) Sickle cell-hemoglobin E disease: clinical findings and implications. Journal of Pediatrics, 119, 949 951.
26. Sanchaisuriya K., Fucharoen G., Sae-ung N., Sae-ue N., Baisungneon R., Jetsrisuparb A. Fucharoen S. (1997) Molecular and hematological characterization of HbE heterozygote with α-thalassemia determinant. South East Asia Journal of Tropical Medicine & Public Health 28 (Suppl. 3 100 103.
27. Schroeder W.A., Powers D., Reynolds R.D. Fisher J.I. (1976-1977) Hb-E in combination with Hb-S and Hb-C in a black family. Hemoglobin 1, 287 289.
28. Vishwanathan C., Agarwal M.B., Bichile L.S. Bhave A.B. (1992) Double heterozygosity for hemoglobin S and E. Indian Pediatrics 29, 895 897.
29. Wasi P., Na-Nakorn S. Pootrakul S. (1974) The α-thalassemias. Clinics in Haematology 3, 384 410.
30. White J.M., Christie B.S., Nam D., Daar S. Higgs D.R. (1993) Frequency and clinical significance of erythrocyte genetic abnormalities in Omanis. Journal of Medical Genetics 30, 396 400.