Thalassemia: Definition, Clinical Features & Complications

Thalassemia is a heterogeneous group of blood disorders affecting the hemoglobin genes and resulting in ineffective erythropoiesis. The decreased production of hemoglobin results in anemia in early age and frequent blood transfusions are required to keep up the hemoglobin levels. This activity outlines the evaluation and treatment of thalassemia and highlights the role of an interprofessional team in managing the patients with this condition.

Objectives:Summarize the etiology of thalassemias. Review of different laboratory and bedside evaluation techniques in the management of thalassemia patients. Outline the treatment and management options available for thalassemia.

Identify interprofessional team strategies for improving care coordination and communication to improve outcomes in thalassemia. IntroductionThalassemias are a heterogeneous grouping of genetic disorders that result from a decreased synthesis of alpha or beta chains of hemoglobin (Hb). Hemoglobin serves as the oxygen-carrying component of the red blood cells.

It consists of two proteins, an alpha, and a beta. If the body does not manufacture enough of one or the other of these two proteins, the red blood cells do not form correctly and cannot carry sufficient oxygen; this causes anemia that begins in early childhood and lasts throughout life. Thalassemia is an inherited disease, meaning that at least one of the parents must be a carrier for the disease.