β- and α-Thalassemia intermedia in Basra, Southern Iraq.

Hemoglobinopathies are common in Iraq and beta-thalassemia major (beta-TM) is a leading health problem in Basra, Southern Iraq. However, beta- and alpha-thalassemia intermedia (beta- and alpha-TI) have not been so well studied. This is a descriptive study of 152 consecutive beta- and alpha-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. beta-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 (beta 4) disease (12.95 +/- 14.8 years), p < 0.05. Patients with beta-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 beta-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 beta-TI. While age is a significant risk factor for osteoporosis in both types of thalassemia intermedia. Both alpha- and beta-thalassemia (alpha- and beta-thal) have been reported in Basra. Although beta-TI is associated with a more severe disease than alpha-TI, both are associated with considerable complications. Thus, genetic studies are needed to determine the types of mutation producing beta-TI and the exact alpha-thal determinants producing Hb H disease as they are important in the prediction of the phenotype severity.