THE HEMOGLOBIN, RDW, AND MEAN CORPUSCULAR VALUES IN PATIENTS WITH BETA-THALASSEMIA/HEMOGLOBIN E DISEASE AND BETA-THALASSEMIA TRAIT

Authors

  • Vinisia Setiadji Clinical Pathology Department, Medical faculty, Universitas Sumatera Utara / Haj Adam Malik Hospital
  • Bidasari Lubis Paediatrics Department, Medical faculty, Universitas Sumatera Utara / Haj Adam Malik Hospital, Medan
  • Adi Koesoema Aman Clinical Pathology Department, Medical faculty, Universitas Sumatera Utara / Haj Adam Malik Hospital, Medan
  • Herman Hariman Clinical Pathology Department, Medical faculty, Universitas Sumatera Utara / Haj Adam Malik Hospital, Medan

DOI:

https://doi.org/10.24293/ijcpml.v25i3.1459

Keywords:

Beta-thalassemia, hemoglobin E, discrepancy, MCV, RDW

Abstract

Beta-thalassemia/hemoglobin E disease is a condition where there is double heterozygosity of beta-thalassemia trait and hemoglobin E trait. This produces a condition with more severe phenotypic appearance compared to beta thalassemia trait and hemoglobin E trait. Logically the Mean Corpuscular Values (MCV) of beta-thalassemia/hemoglobin E disease should also be worsened. The aim of this study was to assess the hemoglobin level, RDW, and MCV between beta-thalassemia/hemoglobin E disease and beta thalassemia trait. The researchers hereby studied eleven cases from two families who were detected to have beta-thalassemia/hemoglobin E disease. Family-1 with beta-thalassemia trait had MCV 68 fL and 65 fL, the MCH value was 21 pg and 20 pg, respectively. In Family-2, mother with beta-thalassemia trait, had MCV 60.2 fL and MCH 18. 8 pg. Daughters with beta-thalassemia/hemoglobin E disease from subjects 1 and 2 whose blood were taken repetitively during visits to the hematology clinic, had mean±SD of MCV 70.8±4.9 fL and Mean Corpuscular Hemoglobin (MCH) value 22.8±2.3 pg. They were significantly higher than the ones with beta-thalassemia trait (p<0.05). Moreover, there were found that the MCV from post-transfusion state were significantly higher than the pre-transfusion state (p<0.001). Based on the study, it could concluded that the MCV from subjects with beta-thalassemia/hemoglobin E disease were persistently higher than the beta-thalassemia trait. The role of blood transfusion in patients with beta-thalassemia/hemoglobin E disease seems to play a part in the result of a discrepancy in this matter.

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References

Fucharoen S and Winichagoon S. Hemoglobinopathies in Southeast Asia. Indian Journal of Medical Research. 2011, 134(4). Page 498-506

Cao A, Galanello R, Rosatelli C. Genotype-Phenotype in Beta Thalassemia. Blood Journal. 1994, vol 8. Page 1-12

Fucharoen, S. Genotype and Phenotype of Thalassemia : A discussion. 2005. Annals New York Academy of Science. Volume 1054. Page 518 – 521

Ricchi P, Filosa A, Maggio A, Fucharoen A. Non-Transfusion-Dependent Thalassemia: A Complex Mix of Genetic Entities yet To Be Fully Discovered. 2015. Biomed Research International. Volume 2015. Page 1-2

Vichinsky E. Hemoglobin E Syndrome. American Society of Hematology. 2007. Page 79-83

Olivieri, N., Pakbaz, Z., Vichinsky, E. HbE / Beta Thalassemia : a common and clinically diverse disorder. 2011. Indian Journal of Medicine, vol 134, pp 522 – 531

Tamam M, Hadisaputro S, Setianingsih A. Hubungan Antara Tipe Mutasi Gen Globin Beta dan Manifestasi Klinis Pada Penderita Thalassemia. Jurnal Kedokteran Briwajaya. Volume 26. Page 48-52

Wahidayat P, Gatot D, Tjitrasari D. Phenotypic Diversity in Beta-HbE Thalassemia Patients. 2006. Pediatrica Indonesia. Volume 6 (3-4). Page 82-86

Modell, B., Darlison, M. Epidemiological estimates for Haemoglobin Disorder : WHO South East Asian rgion by Country. 2007.Modell's Haemoglobinopathologist's Almanac

Sarma PR. In : Walker HK, Hall WD, Hurst JW, editors. Clinical Methods : The History, Physical, and Laboratory Examinations. Boston : Butterworths; 1990. Chapter 152. Page 721-725

Olivieri, N., Muraca, G., O'Donnel, A. Studies in hemoglobin E beta-thalassemia. 2008. British Journal of Hematology. Volume 141. Page 388 – 397

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Submitted

2019-01-30

Accepted

2019-04-04

Published

2019-04-13

How to Cite

[1]
Setiadji, V., Lubis, B., Aman, A.K. and Hariman, H. 2019. THE HEMOGLOBIN, RDW, AND MEAN CORPUSCULAR VALUES IN PATIENTS WITH BETA-THALASSEMIA/HEMOGLOBIN E DISEASE AND BETA-THALASSEMIA TRAIT. INDONESIAN JOURNAL OF CLINICAL PATHOLOGY AND MEDICAL LABORATORY. 25, 3 (Apr. 2019), 343–348. DOI:https://doi.org/10.24293/ijcpml.v25i3.1459.

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