Cytokine dependent hematopoietic cell linker (CLNK) is highly elevated in blood transfusion dependent beta-thalassemia major patients.

BACKGROUND

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 signalling proteins and function in the organisation of the molecular components required for signal transduction.

OBJECTIVES

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.

RESULTS

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.

CONCLUSIONS

Increased CLNK levels in TDT may be explained by reciprocal effects between immune signalling and immature erythrocytes, which release soluble receptors and signalling molecules, including CLNK, in the blood.