β- And α-Thalassemia Intermedia in Basra, Southern Iraq

Abstract

Hemoglobinopathies are common in Iraq and β-thalassemia major (β-TM) is a leading health problem in Basra, Southern Iraq. However, β- and α-thalassemia intermedia (β- and α-TI) have not been so well studied. This is a descriptive study of 152 consecutive β- and α-TI patients registered at the Centre for Hereditary Blood Diseases (CHBD) in Basra, Southern Iraq from October 1 2010 through June 30 2012 including age at diagnosis, blood transfusions and complications. β-Thalassemia intermedia was found in 80 (52.6%) patients with a mean age at diagnosis of 7.10 ± 8.0 years. This was significantly different from that of Hb H (β4) disease (12.95 ± 14.8 years), p <0.05. Patients with β-TI received significantly more blood transfusions (3.39 ± 3.85)/year compared to those with Hb H disease (1.07 ± 1.39)/year, p <0.05.

Short stature, extramedullary erythropoiesis, pulmonary hypertension and iron overload were significantly higher among patients with β-TI compared to those with Hb H disease, p <0.05. Iron overload is a significant risk factor for growth retardation among patients with Hb H and β-TI. While age is a significant risk factor for osteoporosis in both types of thalassemia intermedia.

Both α- and β-thalassemia (α- and β-thal) have been reported in Basra. Although β-TI is associated with a more severe disease than α-TI, both are associated with considerable complications. Thus, genetic studies are needed to determine the types of mutation producing β-TI and the exact α-thal determinants producing Hb H disease as they are important in the prediction of the phenotype severity.

• β-Thalassemia intermedia (β-TI)
• α-Thalassemia intermedia (α-TI)
• Hb H (β4)
• Basra
• Southern Iraq

Acknowledgments

We would like to thank Dr. Sadeq Khalif Ali, a hematologist at the Centre for Hereditary Blood Diseases, Basra, Southern Iraq, for his great help in the laboratory investigations and Professor Assad Yehia, College of Agriculture, Basra, Southern Iraq, for his assistance in the statistical analyses of the data.

Declaration of Interest

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of this article.