Early Screening of Hemoglobinopathy in Indonesia Using Erythrocyte Indices
Abstract
BACKGROUND: The mutation spectrums of hemoglobinopathy are different among populations that yield a different result of erythrocyte indices. Calculation of erythrocyte indices with some formula has been reported to differentiate between hemoglobinopathy and non-hemoglobinopathy, but its cut-off should be recalculated specific for each population to gain a better sensitivity and specificity. We aimed to evaluate red blood cell count (RBC), Mentzer index, red cell distribution width (RDW), RDW index (RDWI), Shine and Lal index (S&L) and Green and King index (G&K) to screen hemoglobinopathy in Indonesia.
METHODS: A retrospective cross-sectional study was performed on 202 subjects. The diagnosis of hemoglobinopathy was determined based on the results of complete blood count (CBC) data, high-performance liquid chromatography (HPLC) and Hemoglobin H (HbH) inclusion body. The ferritin concentration was checked to determine the status of iron. The erythrocytes indices were analyzed and calculated to predict hemoglobinopathy.
RESULTS: A total 202 subjects who met the criteria were involved in this study. Fifty percent showed pure hemoglobinopathy and 4% showed a combination of thalassemia and hemoglobinopathy. The hemoglobin concentration and RBC were significantly higher, and the mean corpuscular volume (MCV) and RDW were significantly lower in hemoglobinopathy compared to iron deficiency. The difference was not significant if the hemoglobinopathy was combined with iron deficiency. By this study's cut-off, the G&K and RDWI showed the highest accuracy, sensitivity, and specificity.
CONCLUSION: The new cut-off of erythrocyte index and its calculation to screen hemoglobinopathy in Indonesia showed a higher sensitivity and specificity, especially for G&K and RDWI with cut-off 73 and 228, respectively. The presence of iron deficiency in hemoglobinopathy could decrease the sensitivity.
KEYWORDS: hemoglobinopathy, RBC, Mentzer index, RDW, RDWI, S&L, G&K
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Bain BJ. Hemoglobinopathy diagnosis. 2nd ed. Massachusetts: Blackwell Publishing; 2006, NLMID.
Batebi A, Pourreza A, Esmailian R. Discrimination of betathalassemia minor and iron deficiency anemia by screening test for red blood cell indices. Turk J Med Sci. 2012; 42: 275-80, article.
Bordbar E, Taghipour M, Zucconi BE. Reliability of different RBC indices and formulas in differentiating between β-thalassemia minor and other microcytic hypochromic cases. Mediterr J Hematol Infect Dis. 2015; 7: e2015022, CrossRef.
Danjou F, Anni F, Galanello R. Beta-thalassemia: from genotype to phenotype. Hematologica. 2011; 96: 1573-5, CrossRef.
DeLoughery TG. Microcytic anemia. T Engl J Med. 2014; 371: 1324-31, CrossRef.
Dirjen Bina Pelayanan Medik, Kementerian Kesehatan Republik Indonesia. Pencegahan Thalassemia (Hasil Kajian HTA Tahun 2009). Jakarta: Kementrian Kesehatan RI; 2010, article.
Janus J, Moerschel S. Evaluation of anemia in children. Am Fam Physician. 2010; 81: 1462-71, PMID.
Khatri A, Kavatkar A, Puranik S. Evaluation of red cell distribution width (RDW) and other indices in microcytic anemias. Med J West India. 2013: 41: 76-80.
Martin A, Thompson AA. Thalassemias. Pediatr Clin N Am. 2013; 60: 1383-91, CrossRef.
Mc Kenzie. Anemia of defective heme synthesis. In: Balado D, editor. Textbook of hematology. 2nd ed. Baltimore: William and Wilkins;1996. p.121-46, NLMID.
Miri-Monghaddam E, Sargolzaie N. Cut off determination of discrimination indices in differential diagnosis between iron deficiency anemia and β-thalassemia minor. Int J Hematol Oncol Stem Cell Res. 2014; 8: 27-32, PMCID.
Mousa AO. Types of anemias with low MCV using Mentzer Index and RBC count among patients seen in Basrah Al-Sadir Teaching Hospital. Med J Babylon. 2014; 11: 292-6, article.
Narchi H, Basal RB. Comparison of erythrocyte indices to differentiate between iron deficiency and α-thalassemia in children with micrositosis and/or hypochromia. East Mediterr Health J. 2010; 9: 966-71, PMCID.
Niazi M, Tahir M, Raziq F, Hameed A. Usefulness of red cell indices in differentiating microcytic hypochromic anemias. Gomal J Med Sci. 2010; 8: 125-9, article.
Nesa A, Tayab A, Sultana T, Khondker L, Rahman Q, Karim A, et al. RDWI is better discriminant than RDW in differentiation of iron deficiency anaemia and beta thalassemia trait. Bangladesh J Child Health. 2009; 33: 100-3, CrossRef.
Okan V, Cigiloglu A, Cifci S, Yilmaz M, Pehlivan M. Red cell indices and functions differentiating patients with the β-thalassaemia trait from those with iron deficiency anaemia. J Int Med Res. 2009; 37: 25-30, CrossRef.
Rahim F, Keikhaei B. Better differential diagnosis of iron deficiency anemia from beta-thalassemia trait. Turk J Hematol. 2009; 26: 138-45, article.
Rathod DA, Kaur A, Patel V, Patel K, Kabrawala R, Patel V, et al. Usefulness of cell counter-based parameters and formulas in detection of β-thalassemia traits in areas of high prevalence. Am J Clin Pathol. 2007; 128: 585-9, CrossRef.
Rund D, Filon D, Strauss N, Rachmilewitz EA, Oppenheim A, et al. Mean corpuscular volume of heterozygotes for beta-thalassemia correlates with the severity of mutations. Blood. 1992; 79: 238-43, PMID.
Sirachainan N, Lamsiriak P, Charoenkwan P, Kadegasem P, Wongwerawattanakoon P, Sasanakul W, et al. New mathematical formula for differentiating thalassemia trait and iron deficiency anemia in thalassemia prevalent area: a study in healthy school-age children. Southeast Asian J Trop Med Public Health. 2014; 45: 174-82, PMID.
Urrechaga E, Aguirre U, Izquierdo S. Multivariable discriminant analysis for the differential diagnosis of microcytic anemia. Anemia 2013: 457834, CrossRef.
Vehapoglu A, Ozgurhan G, Demir AD, Uzuner S, Nursoy MA, Turkmen S, et al. Hematological indices for differential diagnosis in β thalassemia trait and iron deficiency anemia. Anemia. 2014: 576738, CrossRef.
Wang C, Beganyi L, Fernandes BJ. Measurements of red cell parameters in α-thalassemia trait: correlation with the genotype. Lab Hematol. 2000; 6: 163-6.
DOI: https://doi.org/10.18585/inabj.v9i2.313
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