Advertisement

HbE Level and Red Cell Parameters in Heterozygous HbE With and Without α0-Thalassemia Trait

  • Sakorn Pornprasert
  • Monthathip Tookjai
  • Manoo Punyamung
  • Panida Pongpunyayuen
Original Article
  • 35 Downloads

Abstract

We compared hemoglobin (Hb) E levels and red cell parameters between heterozygous HbE with and without α0-thalassemia trait and also determine their appropriated cut-off points for differentiating these two groups. High performance liquid chromatography analysis results and mean levels of red blood cell (RBC) parameters, including RBC count, total Hb, hematocrit, MCV, MCH and MCHC of heterozygous HbE with α0-thalassemia trait (n = 183) and without α0-thalassemia trait (n = 1437) were reviewed and compared. The α0-thalassemia status in these samples was detected by real-time PCR with SYBR Green1 and high resolution melting analysis. Mean levels of HbE, total Hb, MCV, MCH and MCHC of heterozygous HbE with α0-thalassemia trait were significantly lower than those of heterozygous HbE without α0-thalassemia trait (P < 0.001). In addition, HbE level at a cut-off value of < 24% was superior to MCV (< 80 fL) and MCH (< 27 pg) for differentiating the heterozygous HbE with and without α0-thalassemia trait with 100% sensitivity and 87.2% specificity. Despite certain limitations of this study like missing RDW and reticulocyte counts, and not testing for α+-thalassemia and Hb Constant Spring, we conclude that the HbE level at a cut-off point of < 24% is a useful marker for initial discrimination between heterozygous HbE with and without α0-thalassemia trait.

Keywords

α0-Thalassemia Coinheritance HbE High performance liquid chromatography Screening 

Notes

Acknowledgements

The authors thank technicians in the Associated Medical Sciences Clinical Service Center, Faculty of Associated Medical Sciences, Chiang Mai University, Chiang Mai, Thailand for their help and assistance.

Compliance with Ethical Standards

Conflict of interest

The authors report no conflicts of interest.

Ethical Standard

This study was approved by the Ethics Committee of the Faculty of Associated Medical Sciences, Chiang Mai University, Chiang Mai, Thailand.

References

  1. 1.
    Fucharoen G, Fucharoen S, Sanchaisuriya K, Sae-Ung N, Suyasunanond U, Sriwilai P et al (2002) Frequency distribution and haplotypic heterogeneity of beta(E)-globin gene among eight minority groups of northeast Thailand. Hum Hered 53:18–22CrossRefGoogle Scholar
  2. 2.
    Fucharoen S, Winichagoon P (1987) Hemoglobinopathies in southeast Asia. Hemoglobin 11:65–88CrossRefGoogle Scholar
  3. 3.
    Fucharoen S, Winichagoon P (1992) Thalassemia in SouthEast Asia: problems and strategy for prevention and control. Southeast Asian J Trop Med Public Health 23:647–655PubMedGoogle Scholar
  4. 4.
    Traeger J, Wood WG, Clegg JB, Weatherall DJ (1980) Defective synthesis of HbE is due to reduced levels of beta E mRNA. Nature 288:497–499CrossRefGoogle Scholar
  5. 5.
    Fucharoen S, Winichagoon P (2011) Haemoglobinopathies in southeast Asia. Indian J Med Res 134:498–506PubMedPubMedCentralGoogle Scholar
  6. 6.
    Nathalang O, Nillakupt K, Arnutti P, Boonsiri T, Panichkul S, Areekul W (2005) Screening for thalassemia and hemoglobinopathy in a rural area of Thailand: a preliminary study. J Med Assoc Thai 88:35–42Google Scholar
  7. 7.
    Van Delft P, Lenters E, Bakker-Verweij M, de Korte M, Baylan U, Harteveld CL et al (2009) Evaluating five dedicated automatic devices for haemoglobinopathy diagnostics in multi-ethnic populations. Int J Lab Hematol 31:484–495CrossRefGoogle Scholar
  8. 8.
    Charoenkwan P, Wanapirak C, Thanarattanakorn P, Sekararithi R, Sae-Tung R, Sittipreechacharn S et al (2005) Hemoglobin E levels in double heterozygotes of hemoglobin E and SEA-type alpha-thalassemia. Southeast Asian J Trop Med Public Health 36:467–470PubMedGoogle Scholar
  9. 9.
    Leckngam P, Limweeraprajak E, Kiewkarnkha T, Tatu T (2017) The Hb E (HBB: c.79G > A), mean corpuscular volume, mean corpuscular hemoglobin cutoff points in double heterozygous Hb E/- -SEA alpha-thalassemia-1 carriers are dependent on hemoglobin levels. Hemoglobin 41:38–43CrossRefGoogle Scholar
  10. 10.
    Pornprasert S, Wiengkum T, Srithep S, Chainoi I, Singboottra P, Wongwiwatthananukit S et al (2011) Detection of alpha-thalassemia-1 Southeast Asian and Thai type deletions and beta-thalassemia 3.5-kb deletion by single-tube multiplex real-time PCR with SYBR Green1 and high-resolution melting analysis. Korean J Lab Med 31:138–142CrossRefGoogle Scholar
  11. 11.
    Van Vranken M (2010) Evaluation of microcytosis. Am Fam Physician 82:1117–1122PubMedGoogle Scholar
  12. 12.
    Sanchaisuriya K, Chirakul S, Srivorakun H, Fucharoen G, Fucharoen S, Changtrakul Y et al (2008) Effective screening for double heterozygosity of Hb E/alpha0-thalassemia. Ann Hematol 87:911–914CrossRefGoogle Scholar
  13. 13.
    Bianco I, Cappabianca MP, Foglietta E, Lerone M, Deidda G, Morlupi L et al (1997) Silent thalassemias: genotypes and phenotypes. Haematologica 82:269–280PubMedGoogle Scholar
  14. 14.
    Khera R, Singh T, Khuana N, Gupta N, Dubey AP (2015) HPLC in characterization of hemoglobin profile in thalassemia syndromes and hemoglobinopathies: a clinicohematological correlation. Indian J Hematol Blood Transfus 31:110–115CrossRefGoogle Scholar
  15. 15.
    Sharma P, Das R, Trehan A, Bansal D, Chhabra S, Kaur J et al (2015) Impact of iron deficiency on hemoglobin A2% in obligate β-thalassemia heterozygotes. Int J Lab Hematol 37:105–111CrossRefGoogle Scholar

Copyright information

© Indian Society of Hematology and Blood Transfusion 2018

Authors and Affiliations

  1. 1.Department of Medical Technology, Faculty of Associated Medical SciencesChiang Mai UniversityChiang MaiThailand
  2. 2.Associated Medical Sciences Clinical Service Center, Faculty of Associated Medical SciencesChiang Mai UniversityChiang MaiThailand

Personalised recommendations