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Transfusion-dependent β-thalassemia (TDT) is a severe form of thalassemia caused by mutations in the β-globin gene, resulting in partial or complete deficiency of β-globin chains. This deficiency results in oxidative stress, dyserythropoiesis, and chronic anemia. Cytokine-dependent hematopoietic cell linker (CLNK) belongs to adaptor proteins that have the capacity to interact with multiple signaling proteins and function in the organization of the molecular components required for signal transduction.
This is the first study which measured serum CLNK in TDT patients and examines the correlation between CLNK and iron overload biomarkers.
Patients and methods
Sixty children with TDT and 30 normal children (aged 3-12 years old) participated in the present study. The patients were on blood transfusion as a part of their treatment regimen. Serum C-reactive protein was negative in all samples.
The results showed significantly higher (p<0.001) serum CLNK levels in TDT patients as compared with controls. The TDT diagnosis explained 19.4% of the variance in CLNK levels.
The increased levels of CLNK were significantly associated with indicants of iron overload, namely increased ferritin levels.
Increased CLNK levels in TDT may be explained by reciprocal effects between immune signaling and immature erythrocytes, which release soluble receptors and signaling molecules, including CLNK, in the blood.Le texte complet de cet article est disponible en PDF.
Keywords : CLNK, Cytokines, Inflammation, Thalassemia, Ferritin, Iron overload