Dysregulated Serum Cytokine Production in Pediatric Patients with beta-Thalassemia Major

beta-Thalassemia major (beta-TM) is an inherited disorder of hemoglobin (Hb) production, which can cause severe anemia. A compromised immune system has been observed in patients with beta-TM, whereas cytokines have a major role in immune modulation. Interleukin-4 (IL-4), IL-8, IL-13 and transforming growth factor-beta (TGF-beta) are critical in initiating pro-inflammatory responses, and the serum levels of those cytokines may be involved in the pathophysiology of beta-thalassemia (beta-that). To assess this hypothesis, we studied 23 pediatric patients with beta-TM by measuring serum levels of IL-4, IL-8, IL-13 and TGF-beta, as well as evaluating infection frequency per year, total number of transfusions and serum ferritin (SF) levels, together with age-matched healthy controls. We found that patients with beta-that had higher IL-8, IL-13 and TGF-beta concentrations than normal controls, whereas markedly decreased serum IL-4 level was documented in patients with beta-TM. Serum IL-4 level of beta-thal patients showed a negative significant correlation with infection frequency, total number of transfusions and SF levels. On the contrary, serum levels of IL-8, IL-13 and TGF-beta exerted a positive relationship with those clinical parameters. Taken together, our study implies that dysregulated cytokine profile might contribute to iron overloads and impair immune cell functions, thus serving as useful biomarkers for diagnosis and evaluation of beta-TM in the future. Our study sheds new light on the pathogenesis of beta-TM.