Immune dysfunction in beta thalassemia

Immune dysfunction in beta thalassemia

Infectious complications have always been one of the main causes of morbidity and mortality in   β - thalassemia. They have been described as the second most common cause of death in these patients, especially in childhood and adulthood, with a prevalence of 12-13%; cardiac failure and hepatic disease are the first and third most common causes of death, respectively [2,3].

 Besides the well-known risks of blood-borne infections associated with multiple transfusions, a less familiar clinical problem is the increased susceptibility of patients with thalassemia to infections, due to the coexistent immune deficiency. Furthermore, immune abnormalities have been suggested as a precipitating factor for the fourth most common cause of death in β - thalassemia, i.e. malignancies, especially leukemia and lymphomas [3].

The substantial improvement in survival rates and quality of life achieved in β-thalassemia over the last sevberal decades, owing to therapeutic advances and the close follow-up of patients in specialized centers, has redirected the attention of investigators to other collateral abnormalities that were previously neglected or overlooked.