Estimation and correlation of different hemoglobin levels in HbE hemoglobinopathies in Indian population using capillary electrophoresis method.
Keywords:
Capillary electrophoresis, Hemoglobin E (HbE) heterozygote, Hemoglobin E (HbE) homozygote, Compound heterozygote of HbE with Thalassemia.
Abstract
Background: Capillary electrophoresis (CE) estimates Hemoglobin E (HbE) in HbE hemoglobinopathies, which was previously not possible with other method due to combined elution of HbA2 with HbE . Associated hemoglobin abnormalities can be estimated with separation of HbA2 from HbE. Methods: The study is retrospective using CE to detect abnormal HbE and differentiate the HbE syndromes. Student t-test was used for statistical analysis. Result: 119 cases of HbE syndromes were identified and divided into HbE heterozygotes (71), HbE heterozygotes with borderline HbA2 (15), compound heterozygotes of HbE with Thalassemia (13 HbE with β-Thalassemia / 8 HbE with α-Thalassemia), compound heterozygotes of HbE with HbS (HbSE) (2) and HbE homozygotes (10). Mean HbA2 shows increasing pattern with increasing severity of HbE syndrome. However, compound heterozygote of HbE and β-Thalassemia (HbE-β-Thal) shows maximum mean level of HbA2 (5.46%). HbA2 of HbE heterozygote and HbE heterozygote with borderline HbA2 was not found to be significantly different, statistically. Fetal haemoglobin (HbF) of HbE homozygotes is found significantly higher than that of HbE heterozygotes, but significantly lower than that of HbE-Thalassemia. The HbE values of HbE with α-Thalassemia (HbE-α-Thal) and HbE-β-Thal were found to be below -3SD value (14.77%) and beyond +3SD value (37.77%) of mean of HbE of HbE heterozygote (26.27%), respectively.Conclusion: The study identifies range of different hemoglobin levels in HbE syndromes, with special reference to HbA2. Concurrent iron deficiency anaemia also needs to be kept in mind in dealing with a population where iron deficiency is very common. DOI: 10.21276/APALM.1228References
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15. Cotton F, Malaviolle X, Vertongen F, Gulbis B. Evaluation of an automated capillary electrophoresis system in the screening for hemoglobinopathies. Clin Lab 2009; 55 (5-6): 217-221
16. Cotton F, Wolff F, Gulbis B. Automated capillary electrophoresis in the screening for hemoglobinopathies. Methdods Mol Biol 2013; 984: 227-23
17. Keren DF, Hedstrom D, Gulbranson R, Ou C, Bak R. Comparison of S ebia Capillarys Capillary Electrophoresis With the Primus High – Pressure Liquid Chromatography in the Evaluation of Hemoglobinopathies. Am J Clin Path 2008; 130: 824-831
18. Winichagoon P, Svasti S, Munkongdee T, et al. Rapid diagnosis of thalassemias and other hemoglobinopathies by capillary electrophoresis. Transl Res. 2008; 152: 178-184
19. Rao S, Kar R, Gupta SK, Chopra A, Saxena R. Spectrum of hemoglobinopathies diagnosed by cation exchange HPLC & modulating effects of nutritional deficiency anaemias from north India. Indian J Med Res 2010; 132: 513-519
20. Denic S et al. Hemoglobin A2 Lowered by Iron Deficiency and 𝛼 - Thalassemia: Should Screening Recommendation for 𝛽 - Thalassemia Change ? ISRN Hematol 2013: 1-5
21. Lim WF, Muniandi L, George E, Sathar J, The LK, Lai MI. HbF in HbE / β -thalassemia: A clinical and laboratory correlation. Hematol 2015; 20 (6): 349-353
22. Charoenkwan P, Wanapirak C, Thanarattanakorn P, Sekararithi R, Sae - Tung R, Sittipreechacharn S, Sanguansermsri T. Hemoglobin E levels in double heterozygotes of hemoglobin E and SEA - type alpha - Thalassemia. Southeast Asian J Trop Med Public Health 2005; 36 (2): 467-70
23. Hafiza A, Malisa MY, Khirotdin RD, Azlin I, Azma Z, Thong MC et al. HbA2 levels in normal, beta - Thalassaemia and hemoglobin E carriers by capillary electrophoresis. Malays J Pathol. 2012; 34 (2): 161-4
2. Aggarwal S, Saluja S, Bhasin S, Sharma M, Gupta DK, Gupta B, Mittal V. HbE variants – Retrospective analysis in a tertiary care centre. J Indian Acad Clin Med 2011; 12(4): 263-265
3. Tyagi S, Pati HP, Choudhary VP, Saxena R. Clinico - heemotological profile of HbE syndrome in adults and children. Haematology 2004; 9: 57-60
4. Deka R, Gogoi BC, Hundrieser J, et al. Hemoglobinopathies in northeast India. Hemoglobi. 1987; 11 :531-538
5. Singh RM, Choudhury B, Singh TS. Hemoglobin E distribution in four endogamous populations of Manipur (India). Eurasian J Anthropol 2010: 1(2): 109-117
6. Mais DD, Gulbranson RD, Keren DF. The range of hemoglobin A2 in Hemoglobin E heterozygotes as determined by capillary electrophoresis. Am J Clin Pathol 2009; 132: 34-38
7. Vichinsky E. Hemoglobin E syndromes. Hematology Am Soc Hematol Educ Program 2007: 79-83
8. Premawardhena A, Fisher CA, Olivieri NF, et al. Hemoglobin E β Thalassaemia in Sri Lanka. Lancet 2005; 366: 1467-1470.
9. Mishra P, Pati HP, Chatterjee T, et al. Hb SE disease: a clinic – hematological profile. Ann Hematol 2005; 84: 667-670
10. Greer JP et al .editors. Wintrobe’s Clinical Hematology. 12th ed. Philadelphia: Lippincott Williams & Wilkins; 2009
11. Schroeder WA, Shelton JB, Shelton JR et al. The estimation of HbA2 in the presence of HbC or HbE by reverse phase high performance liquid chromatography. Hemoglobin 1986; 10: 253-257
12. Keren DF, Hedstrom D, Gulbranson R et al. Comparison of Sebia Capillarys electrophoresis with the Primus high - pressure liquid chromatography in the evaluation of hemoglobinopathies. Am J Clin Pathol 2008; 130: 824-831
13. Cotton F, Changying L, Fontaine B et al. Evaluation of a capillary electrophoresis method for routine determination of hemoglobins A2 and F. Clin Chem. 1999; 45: 237-243
14. Jenkins MA, Hendy J, Smith IL. Evaluation of hemoglobin A2 quantification assay and hemoglobin variant screening by capillary electrophoresis. J Capillary Electrophor 1997; 4: 137-143
15. Cotton F, Malaviolle X, Vertongen F, Gulbis B. Evaluation of an automated capillary electrophoresis system in the screening for hemoglobinopathies. Clin Lab 2009; 55 (5-6): 217-221
16. Cotton F, Wolff F, Gulbis B. Automated capillary electrophoresis in the screening for hemoglobinopathies. Methdods Mol Biol 2013; 984: 227-23
17. Keren DF, Hedstrom D, Gulbranson R, Ou C, Bak R. Comparison of S ebia Capillarys Capillary Electrophoresis With the Primus High – Pressure Liquid Chromatography in the Evaluation of Hemoglobinopathies. Am J Clin Path 2008; 130: 824-831
18. Winichagoon P, Svasti S, Munkongdee T, et al. Rapid diagnosis of thalassemias and other hemoglobinopathies by capillary electrophoresis. Transl Res. 2008; 152: 178-184
19. Rao S, Kar R, Gupta SK, Chopra A, Saxena R. Spectrum of hemoglobinopathies diagnosed by cation exchange HPLC & modulating effects of nutritional deficiency anaemias from north India. Indian J Med Res 2010; 132: 513-519
20. Denic S et al. Hemoglobin A2 Lowered by Iron Deficiency and 𝛼 - Thalassemia: Should Screening Recommendation for 𝛽 - Thalassemia Change ? ISRN Hematol 2013: 1-5
21. Lim WF, Muniandi L, George E, Sathar J, The LK, Lai MI. HbF in HbE / β -thalassemia: A clinical and laboratory correlation. Hematol 2015; 20 (6): 349-353
22. Charoenkwan P, Wanapirak C, Thanarattanakorn P, Sekararithi R, Sae - Tung R, Sittipreechacharn S, Sanguansermsri T. Hemoglobin E levels in double heterozygotes of hemoglobin E and SEA - type alpha - Thalassemia. Southeast Asian J Trop Med Public Health 2005; 36 (2): 467-70
23. Hafiza A, Malisa MY, Khirotdin RD, Azlin I, Azma Z, Thong MC et al. HbA2 levels in normal, beta - Thalassaemia and hemoglobin E carriers by capillary electrophoresis. Malays J Pathol. 2012; 34 (2): 161-4
Published
2017-08-31
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