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Abstract
Thalassemia is a genetic disorder caused by impaired synthesis of globin chains in hemoglobin (Hb) molecule which can be partial or total. Since thalassemia is inherited from parents to their children, screening is compulsory to prevent newborn at risk from major thalassemia. The incidence thalassemia cases can be found around the world but majority cases emerge among Mediterranean, easter, and Asian. Thalassemia minor is diagnosed based on erythrocyte indices and confirmed by molecular analysis of Hb and DNA. Currently, several formulas are developed to identify the existence of thalassemia. This cross sectional study was aimed to know how good the formulas is. Thalassemia screening was done to 138 donors with minimum of Hb 12,5 g/dL. Detection and identification proses was conducted by the use of calculation index formulas for erythrocytes namely Mentzer index, Srivastava, Shine & Lal, and England & Fraser. The results showed that all of four indexes can detect ? thalassemia minor. The highest degree of sensitivity is Mentzer and Shine & Lal indexs (100%), whereas the highest specificity is Srivastava index (100%). In addition, ? thalassemia and Hb variant (HbE) can also be detected, although not 100%. This result shows that the formulas can be used as a screening of thalassemia, especially in areas those have no access
to molecular laboratory.
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References
-
Ahmed, N., Dawson, M., Smith, C., & Wood. 2007. Biology of disease, T & F informa, UK.
Au W.Y, & Liang, R. 2007. 'Thalassemia management in Hong Kong', ISBT science series, vol.2, p:160-166.
Bain, B.J. 2006. Blood cells a practical guide. 4th ed., Australia: Blackwell publishing.
Higgs, D.R., Olivieri, N.F., & Weatherall, D.J. 2009, Disorders of hemoglobin, Genetics, Patophysiology, and clinical management 2nd ed, eds. Steinberg MH, Forget BG, Higgs DR, & Weatherall DJ, Cambridge University Press, UK.
Judd, S. J. 2010. Health reference series, genetic disorders sourcebook. 4th ed. USA: Omniographics.
Fucharoen S., et.al. 1998. Prenatal and postnatal diagnoses of thalasemias and hemoglobinopathies by HPLC. Clinical chemistry, Vol.44, No.4 :740-747.
Kohne, E. 2011. Hemoglobinopaties clinical manifestation, diagnosis, and treatment. Dtsch Arztebl Int, Vol.108, No.31-32 : 532-540.
Muncie, H. L., & Campbell, J. S. 2009. alpha and beta thalassemia. American family physician, vol.80, no.4, p: 339-344.
Niazi, M., Tahir, M., Raziq, F.E. & Hameed, A. 2010. Usefulness of red cell indices in differentiating microcytic hypochromic anemias. Gomal Journal of Medical Sciences, vol.8, no.2, p:.125-129.
Rathod, D.A., et.al. 2007. Usefulnes of cell counter-based and formulas in detection of ?-thalasemia trait in areas of high prevalence. American society for clinical pathology, vol.128, p:.585-589.
Rogers, K. 2011. The human body, blood physiology and circulation, New York USA : Britannica educational publishing.
Sacher, R.A., & McPherson, R.A. 2004. Tinjauan klinis hasil pemeriksaan laboratorium.Ed. Hartanto, H. Jakarta: EGC.