Hematological and Biochemical status of Beta thalassemia in Pakistani and Afghani patients of Quetta city, Pakistan

Paper Details

Research Paper 01/12/2019
Views (248) Download (11)

Hematological and Biochemical status of Beta thalassemia in Pakistani and Afghani patients of Quetta city, Pakistan

Asma Abdul Ghani, Asmat Ullah Kakar, Zafar Ullah, Inayat Ullah, Azmat Ullah, Mir Chakar Baloch, Mahrukh Naseem
Int. J. Biosci.15( 6), 283-289, December 2019.
Certificate: IJB 2019 [Generate Certificate]


Thalassemia is a genetic blood disorder in which body is unable to synthesis hemoglobin characterized by chronic anemia. Improper erythropoiesis is the major problem in thalassemia. 50 Pakistani and 50 Afghani patients (male and female) from various public sector hospitals of Quetta city a year, were include in this study. Patients were divided into four groups (N=25 each group): Group-I: Included male and female Pakistani control individuals, Group-II Included male and female Pakistani thalassemia patients, Group-III included male and female Afghan control individuals, Group-IV included male and female Afghan thalassemia patients. BMI (Kg/m2) was recorded. 3-5 ml of blood was collected, serum was isolated and biochemical analysis for hematological parameters (Hb, MCV, MCH, MCHC, and PCV), renal function test (urea, creatinine) and liver function test (AST, ALT) were done.  A significant reduction in the BMI (P<0.0001) was reported both in Pakistani and Afghani thalassemia patients as compared to normal individuals. Significant decrease was found for hematological parameters in thalassemia patients in both populations and in both genders. Serum AST, ALT, creatinine and urea was significant increase in both Pakistani and Afghani thalassemia patients as compare to control. The prevalence of thalassemia is more severe in Afghani patients as compare to Pakistani patients since in Afghanistan health facilities are very poor, inter tribe marriages are very common and lack of knowledge.


Ayyash H, Sirdah M. 2018. Hematological and biochemical evaluation of β-thalassemia major (βTM) patients in Gaza Strip: A cross-sectional study. International Journal of Health Sciences 12, 18.

Bekhit OE, El Dash HH, Ahmed MS. 2017. Early detection of kidney dysfunction in Egyptian patients with beta-thalassemia major. Egyptian Pediatric Association Gazette 65, 85-89. https://doi.org/10.1016/j.epag.2017.02.002

Cebrian FY, Flores MVR, Álvarez SI, Salinas IP, Iturrate CR. 2016. Combination of a triple alpha-globin gene with beta-thalassemia in a gypsy family: importance of the genetic testing in the diagnosis and search for a donor for bone marrow transplantation for one of their children. BMC Research Notes 9, 220. https://doi.org/10.1186/s13104-016-2027-1.

Chutvanichkul B, Vattanaviboon P, Masoodi S, Upratya Y, Wanachiwanawin W. 2018. Labile iron pool as a parameter to monitor iron overload and oxidative stress status in β‐thalassemic erythrocytes. Cytometry Part B: Clinical Cytometry 94(4), 631-636. https://doi.org/10.1002/cyto.b.21633

Delvecchio M, Cavallo L. 2010. Growth and endocrine function in thalassemia major in childhood and adolescence. Journal of Endocrinological Investigation 33, 61-68.

Faruqi A. 2014. Association of Serum Ferritin Levels with Haematological Parameters in Thalassaemia Major Patients. Journal of Rawalpindi Medical College 18, 219-221.

Ferdaus MZ, Hasan A, Shekhar HU. 2010. Analysis of serum lipid profiles, metal ions and thyroid hormones levels abnormalities in β-thalassaemic children of Bangladesh. JPMA. The Journal of the Pakistan Medical Association 60, 360.

Galanello R, Origa R. 2010. Beta-thalassemia. Orphanet Journal of Rare Diseases 5, 11.

Galanello R, Perseu L, Satta S, Demartis F, Campus S. 2011. Phenotype-genotype correlation in β-thalassemia. Thalassemia Reports 1, e6-e6. https://doi.org/10.4081/thal.2011.s2.e6

Greene DN, Vaughn CP, Crews BO, Agarwal AM. 2015. Advances in detection of hemoglobinopathies. Clinica Chimica Acta 439, 50-57. https://doi.org/10.1016/j.cca.2014.10.006

Hagag AA, Elfrargy MS, Elfatah MA, El-Lateef A. 2014. Comparative Study of Deferiprone and Silymarin versus Deferiprone and Placebo as Iron Chelators in Children with Beta Thalassemia with Iron Overload. Journal of Leukemia 2, 2. http://dx.doi.org/10.4172/2329-6917.1000130.

Hosen MB, Karmokar NC, Karim MF, Al Mahmud R, Mesbah M. 2015. Association of AST, ALT, ALB and Total Protein with Beta-thalassemia in Bangladeshi Population. International Journal 3, 991-995.

Karim MF,  Ismail M,  Hasan AM, Shekhar HU. 2016. Hematological and biochemical status of Beta-thalassemia major patients in Bangladesh: A comparative analysis. International Journal of Hematology-Oncology and Stem Cell Research 10, 7.

Koliakos G, Papachristou F, Koussi A, Perifanis V, Tsatra I, Souliou E, Athanasiou M. 2003. Urine biochemical markers of early renal dysfunction are associated with iron overload in beta-thalassaemia. Clinical and Laboratory Hematology 25(2), 105-109. https://doi.org/10.1046/j.13652257.2003.00507.x

Lai MI, Jiang J, Silver N, Best S, Menzel S, Mijovic A, Collele S, Ragoussis J, Garner C, Weiss MJ, Thein SL. 2006. α-hemoglobin stabilizing protein is a quantitative trait gene that modifies the phenotype of β-thalassemia. British Journal of Haematology 133, 675-682. https://doi.org/10.1111/j.13652141.2006.06075.x

Maheen H, Malik F, Siddique B, Qidwai A. 2015. Assessing parental knowledge about thalassemia in a thalassemia center of Karachi, Pakistan. Journal of Genetic Counseling 24, 945-951.

Maleknejad S, Heidarzadeh A, Ghandi Y. 2009. Urine β2 Microglobulin and other biochemical Indices in β Thalassemia Major. Acta Medica Iranica 1, 443-446.

Mansi K, Aburjai T, AlBashtawy M, Abdel-Dayem M. 2013. Biochemical factors relevant to kidney functions among Jordanian children with beta-thalassemia major treated with deferoxamine. International Journal of Medicine and Medical Sciences 5, 374-379. https://doi.org/10.5897/IJMMS12.003.

Nasr MR, Ebrahim NA, Salahedin O. 2012. Growth pattern in children with beta-thalassemia major and its relation with serum ferritin, IGF1 and IGFBP3. Journal of Clinical and Experimental Investigations 3, 157-163.

Noetzli LJ, Panigrahy A, Mittelman SD, Hyderi A, Dongelyan A, Coates TD, Wood JC. 2012. Pituitary iron and volume predict hypogonadism in transfusional iron overload. American Journal of Hematology 87, 167-171. https://doi.org/10.1002/ajh.22247

Pilon AM, Nilson DG, Zhou D, Sangerman J, Townes TM, Bondine DM, Gallagher PG. 2006. Alterations in expression and chromatin configuration of the alpha hemoglobin-stabilizing protein gene in erythroid Krüppel-like factor-deficient mice. Molecular and Cellular Biology 26, 4368-4377. https://doi.org/10.1128/MCB.02216-05

Quinn CT, Johson VL, Kim HY, Trachtenberg F, Vogiatzi MG, Kwiatkowski JL, Fung E, Oliveri N, Kirby M, Giardina PJ. 2011. Renal dysfunction in patients with thalassaemia. British Journal of Haematology 153, 111-117. https://doi.org/10.1111/j.13652141.2010.08477.

Risoluti R, Materazzi S, Sorrentino F, Bozzi C, Caprari P. 2018. Update on thalassemia diagnosis: new insights and methods. Talanta 183, 216-222. https://doi.org/10.1016/j.talanta.2018.02.071

Rujito L, Sasongko TH. 2018. Genetic Background of β Thalassemia Modifier: Recent Update. Journal of Biomedicine and Translational Research 4, 12-21. https://doi.org/10.14710/jbtr.v4i1.2541

Rund D, Rachmilewitz E. 2005. β-Thalassemia. New England Journal of Medicine 353, 1135-1146. https://doi.org/10.1056/NEJMra050436

Salama KM, Ibrahim OM, Kaddah AM, Boseila S, Abu Ismail L, Abdel Hamid MM. 2015. Liver Enzymes in Children with beta-Thalassemia Major: Correlation with Iron Overload and Viral Hepatitis. OA Maced Journal of Medical Scincies 3(2), 287-292. http://dx.doi.org/10.3889/oamjms.2015.059

Saral N, Rathore M, Bohra V, Gupta M. 2015. Diagnostic significance of Liver and Renal function tests (LFT&RFT) in Iron overload in patients with β Thalassemia major. International Journal of Clinical Biochemistry and Research 2, 27-32.

Schoorl M, Schoorl M, Van Pelt J, Bartels PC. 2015. Application of innovative hemocytometric parameters and algorithms for improvement of microcytic anemia discrimination. Hematology Reports 1, 7. https://doi.org/10.4081/hr.2015.5843

Taher A, Isma’eel H, Cappellini MD. 2006. Thalassemia intermedia: revisited. Blood Cells Molecules and Diseases 37, 12-20. https://doi.org/10.1016/j.bcmd.2017.06.001

Thein SL. 2018. Molecular basis of β thalassemia and potential therapeutic targets. Blood Cells Molecules and Diseases 70, 54-65. https://doi.org/10.1016/j.bcmd.2017.06.001

Theodorou A, Phylactides M, Forti L, Cramarossa MR, Spyrou P, Gambari R, Thein SW, Kleanthous M. 2016. The investigation of resveratrol and analogs as potential inducers of fetal hemoglobin. Blood Cells Molecules and Diseases 58, 6-12.

Yuksel IO, Koklu E, Kurtoglu E, Arslan S, Cagirci G, Karakus V, Kus G, Cay S, Kucukseymen S. 2016. The association between serum ferritin level, tissue Doppler echocardiography, cardiac T2* MRI, and heart rate recovery in patients with beta thalassemia major. Acta Cardiologica Sinica 32, 231. https://doi.org/10.6515/ACS20150824A