PATTERN OF THALASSEMIAS AND OTHER HEMOGLOBINOPATHIES: A STUDY IN DISTRICT DERA ISMAIL KHAN, PAKISTAN

Abstract

Background: Thalassemia is used to describe disorders with a significant decrease in the rate of synthesis of
a globin chain. The term hemoglobinopathy is used to indicate those disorders with structurally abnormal hemoglobin.
The study was aimed to see the pattern of different hemoglobin disorders in referred cases of district
Dera Ismail Khan and adjacent area.
Material & Methods: This study was carried out on 300 patients who were referred for the Hemoglobin electrophoresis
from October 2009 to April 2013. Detailed clinical history regarding age, sex, cast, family history, blood
transfusion history and physical findings like splenomegaly were noted. All blood samples were analyzed for Hb
Electrophoresis in the Department of Pathology, Gomal Medical College Dera Ismail Khan, Pakistan.
Results: Out of total 300 referred cases, 227 (75.6%) were detected to have abnormal hemoglobin. Among
hemoglobin disorders the most common disorder was Beta thalassemia major in 87(38.30%) followed by sickle
cell disease 73(32.16%) and Beta thalassemia trait 42 (18.5%). Other hemoglobin disorders like Beta thalassemia
intermedia, sickle cell trait, sickle cell/beta thalassemia, HbE disorder were collectively detected in 25 (11.0%)
cases.
Conclusion: Hemoglobin disorders are still a problem for the different communities of Pakistan. Preventive measures
like pre-marriage detection of carrier state must be considered as mandatory step to control the disease.