Is There a Relationship Between CXCR4 Gene Expression and Prognosis of Immune Thrombocytopenia in Children?

Abstract

Immune thrombocytopenia (ITP) is a common autoimmune disorder characterized by decreased platelet count (thrombocytopenia) and bleeding symptoms due to production of autoantibodies against platelets. Chemokines are molecules inducing chemotaxis and play an important role in megakaryopoiesis, including CXCR4 chemokine receptor. CXCR4 is expressed on cells of megakaryocytic series, especially platelets, and triggers several mechanisms in these cells. The purpose of this study was to evaluate the pattern of CXCR4 gene changes upon diagnosis and after treatment and its comparison with laboratory findings in peripheral blood samples from newly diagnosed ITP patients. 35 newly diagnosed patients with ITP and 35 healthy controls were enrolled in this study. CXCR4 gene expression was investigated before and after treatment using real-time PCR. HPRT gene was used as the reference gene to calculate the expression rate of CXCR4 as CXCR4/HPRT ratio. CXCR4 gene expression upon diagnosis and after treatment in peripheral blood plasma of ITP patients showed a significant decrease in comparison with the control group while its expression did not change before and after treatment. No significant correlation was found between the expression of this gene and laboratory parameters. Due to unpredictable course of ITP in patients and the possibility of its progress to refractory form, accurate choice of a biomarker is essential for evaluating prognosis and detection of resistant forms.

Cellular expression of CD markers in immune thrombocytopenic purpura: implications for prognosis

“…Immune thrombocytopenic purpura is an autoimmune disorder that is associated with the peripheral destruction of platelets by autoantibodies against platelet GPs . The pathophysiology of ITP is heterogeneous and genetic background (such as the percentage of polymorphisms in immune system‐related genes), as well as aberrant expressions of some chemokine receptors and micro‐RNAs alongside antiplatelet antibodies, may play a crucial role in predisposition to ITP and even response to treatment . Moreover, impairment of the number and function of different immune cells can play an important role in the initiation and prolongation of ITP .…”

Expression of CD markers' in immune thrombocytopenic purpura: prognostic approaches

“…Studies show that the chemokine receptors VLA‐4 (CD49d), CX3CR1 (CD181), and CXCR4 (CD184) are also increased on CTL of ITP patients . These receptors bind their ligands and cause CTLs shift from PB to BM, which leads to the recruitment of CTLs for platelet destruction and inhibition of megakaryocyte maturation in BM of patients, resulting in reduced platelet counts (Table ) . In ITP patients, it has been reported that increased IL‐27 levels, which causes decreased granzyme B production in CTL, reduces the platelet destruction and enhances the response to treatment of these patients .…”

Re-evaluate the Prognostic Value of Absolute Lymphocyte Count in Pediatric Immune Thrombocytopenia

“…[4][5][6] Therefore, it is important to predict the prognosis early so that treatment modification can be prepared. [7][8][9][10] Recently a meta-analysis 11 including several studies suggested that the levels of absolute lymphocyte count (ALC) were associated with the development of chronic ITP. However, it is well known that in childhood, the ALC levels vary with age.…”

“…Prolonged disease course and long-term use of steroid inevitably affect children’s growth and bring great psychological burden to their families 4–6. Therefore, it is important to predict the prognosis early so that treatment modification can be prepared 7–10. Recently a meta-analysis 11 including several studies suggested that the levels of absolute lymphocyte count (ALC) were associated with the development of chronic ITP.…”