A Long Term Screening of Iranian Populations with Thalassemia and Hemoglobinopathies

摘要: Background : Thalassemias and hemoglobinopathies are genetic autosomal recessive disorders affecting hemoglobin molecules in different ways, qualitatively quantitatively. The highest prevalence of thalassemia has been reported the belt Mediterranean region countries Middle East including Iran. present study evaluated α/ β-thalassemias a large number Iranian populations. Methods: This five-year was conducted on 3780 individuals. Initially complete blood cell count, HPLC, Hb electrophoresis HbA 2 measurement for carrier identification. MCV≤80fL, MCH˂27pg Hb-A2>3.5% were standard diagnostics β-thalassemia diagnosis. In cases with low MCV MCH indices (MCV≤80 fl, MCH<27 pg) Hb-A2< 3.5% normal Hb-electrophoresis, α-thalassemia considered list differential Patients abnormal varieties candidate more precise analysis HPLC. exclusion iron deficiency serum ferritin also measured. Results: Our results revealed that 1932 (51.11%) had electrophoretic pattern, 781 (20.66%) trait 487 (12.84%) major or intermedia, 328 (8.67%) along 142 (3.75%) -electrophoresis status but indices. We identified 11 (0.29%) Alpha variants Hb-H disease/alpha 22 (o.58%) sickle 18(o.47%) disease 9 (0.23%) HbS-Thalassemia double heterozygote 5 (0.13%) E- 32 (0.84%) Hb-D variant 1 (0.026%) Hb-C Iran Hb-J HbS/D heterozygote, Hb-D/J (0,026%) Hb-constant spring/HB-H heterozygote. Conclusion: is common β-globin gene defect among HPLC fast reliable method clinical laboratories especially diagnosis rare as an important auxiliary tool thalassemia.