Clinically, beta thalassemia intermedia is midway in severity between major and minor. Growth and development in children with this disorder can usually be considered normal and most patients have a normal life span; however, some patients have facial bone deformity and/or splenomegaly. Hemoglobin levels are usually decreased with a disproportionately high red blood cell count.
Transfusions (again with iron-chelating agents) may be used as a supportive therapy if necessary.
Beta thalassemia is attributed to a wide variety of genotypes including B+/B+, B0/B+, or B0/B. All of the genotypes have in common a significant reduction in production of the beta-globin chain, with subsequent reduction in the quantity of HbA.
