α⁰-Thalassemia and Related Disorders in Northeast Thailand: A Molecular and Hematological CharacterizationSae-ung N. · Fucharoen G. · Sanchaisuriya K. · Fucharoen S.
Centre for Research and Development of Medical Diagnostic Laboratories, Faculty of Associated Medical Sciences, Khon Kaen University, Khon Kaen, Thailand
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Article / Publication Details
α⁰-Thalassemia is the most severe form of α-thalassemia commonly encountered in Asians. To provide relevant information for effective prevention and control of this disorder, we have examined the molecular basis and hematological features of α⁰-thalassemia-related disorders in northeast Thailand. A multiplex polymerase chain reaction for simultaneous detection of the southeast Asian (SEA) and the THAI α⁰-thalassemia determinants was developed and used for screening of 1,541 Thai individuals who were positive at the preliminary screening in an ongoing thalassemia control program. α⁰-Thalassemia deletions were detected in 397 (25.8%) cases, 396 with the SEA deletion and 1 with the THAI deletion. While the latter was found in association with the Hb Constant Spring in a patient with severe Hb H disease, the former was encountered in as many as 12 thalassemia genotypes whose hematological features were comparatively presented. The results obtained should prove useful in carrier screening and prenatal diagnosis programs of this common genetic disorder in the region.
© 2007 S. Karger AG, Basel
Fucharoen S, Winichagoon P: Hemoglobinopathies in Southeast Asia. Hemoglobin 1987;11:65–88.
- Lau YL, Chan LC, Chan AYY, Ha SY, Yeung CY, Waye JS, Chui DHK: Prevalence and genotypes of α- and β-thalassemia carriers in Hong Kong: implications for population screening. N Engl J Med 1997;336:1298–1301.
- Chui DHK, Waye JS: Hydrops fetalis caused by α-thalassemia: an emerging health care problem. Blood 1998;91:2213–2222.
- Fucharoen S, Winichagoon P, Sirithanaratkul N, Chowthaworn J, Pootrakul P: α-and β-thalassemia in Thailand. Ann NY Acad Sci 1998;850:412–414.
- Boonsa S, Sanchaisuriya K, Fucharoen G, Wiangnon S, Jetsrisuparb A, Fucharoen S: The diverse molecular basis and hematological features of Hb H and AEBart’s diseases in northeast Thailand. Acta Haematol 2004;111:149–154.
- Fucharoen G, Sanchaisuriya K, Sae-ung N, Dangwibul S, Fucharoen S: A simplified strategy for thalassaemia and haemoglobin E in rural communities in south-east Asia. Bull World Health Organ 2004;82:364–372.
- Sanchaisuriya K, Fucharoen S, Fucharoen G, Ratanasiri T, Sanchaisuriya P, Changtrakul Y, Ukosanakarn U, Ussawaphark W, Schelp FP: A reliable screening protocol for thalassemia and hemoglobinopathies in pregnancy: an alternative approach to electronic blood cell counting. Am J Clin Pathol 2005;123:113–118.
Weatherall DJ, Clegg JB: The Thalassaemia Syndrome, ed 4. Oxford, Blackwell Scientific Publication, 2001.
- Fucharoen G, Fucharoen S: Rapid and simultaneous non-radioactive method for detecting α-thalassemia 1 (SEA type) and Hb Constant Spring genes. Eur J Haematol 1994;53:186–187.
- Fucharoen S, Fucharoen G, Sanchaisuriya K, Pengjam Y: Molecular analysis of a Thai β-thalassemia heterozygote with normal haemoglobin A2 level: implication for population screening. Ann Clin Biochem 2002;39:44–49.
- Fucharoen S, Fucharoen G, Fukumaki Y: Simple non-radioactive method for detecting hemoglobin Constant Spring gene. Lancet 1990;335:1527.
- Fucharoen S, Fucharoen G, Ratanasiri T, Jetsrisuparb A, Fukumaki Y: A simple non-radioactive assay for hemoglobin E gene in prenatal diagnosis. Clin Chim Acta 1994;229:197–203.
- Sanchaisuriya K, Fucharoen G, Fucharoen S: Hb Paksé [(α2)codon142 (TAA→TAT) or (Term→Tyr)] in Thai patients with EABart’s disease and Hb H disease. Hemoglobin 2002;26:227–235.
- Eng B, Patterson M, Borys S, Chui DH, Waye JS: PCR base diagnosis of the Filipino (- - FIL) and Thai (- - THAI) α-thalassemia-1 deletions. Am J Hematol 2000;63:54–56.
- Panyasai S, Sringam P, Fucharoen G, Sanchaisuriya K, Fucharoen S: A simplified screening for α-thalassemia 1 (SEA type) using a combination of a modified osmotic fragility test and a direct PCR on whole blood cell lysates. Acta Haematol 2002;108:74–78.
- Sanchaisuriya K, Fucharoen G, Sae-ung N, Jetsrisuparb A, Fucharoen S: Molecular and hematologic features of hemoglobin E heterozygotes with different forms of α-thalassemia in Thailand. Ann Hematol 2003;82:612–616.
- Fucharoen G, Trithipsombat J, Sirithawee S, Yamsri S, Changtrakul Y, Sanchaisuriya K, Fucharoen S: Molecular and hematological profiles of hemoglobin EE disease with different forms of α-thalassemia. Ann Hematol 2006;85:450–454.
- Huisman THJ: Hb E and α-thalassemia; variability in the assembly of βE chain containing tetramers. Hemoglobin 1997;21:227–236.
- Siriratmanawong N, Fucharoen G, Sanchaisuriya K, Ratanasiri T, Fucharoen S: Simultaneous PCR detection of β-thalassemia and α-thalassemia 1 (SEA type) in prenatal diagnosis of complex thalassemia syndrome. Clin Biochem 2001;34:377–380.
- Thein SL: Genetic insights into the clinical diversity of β thalassaemia. Br J Haematol 2004;124:264–274.
- Viprakasit V, Tanphaichitr VS: Compound heterozygosity for α0-thalassemia (- - THAI) and Hb Constant Spring causes severe Hb H disease. Hemoglobin 2002;26:155–162.
- Charoenkwan P, Taweephon R, Sae-Tung R, Thanarattanakorn P, Sanguansermsri T: Molecular and clinical features of Hb H disease in northern Thailand. Hemoglobin 2005;29:133–140.
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