الفهرس | Only 14 pages are availabe for public view |
Abstract Background: serum soluble cytotoxic t-lymphocyte-associated antigen 4 (sCTLA-4) exerts either inhibitory or stimulatory effect depending on the activation state of the immune response. Several studies reported the observation of elevated levels of sCTLA-4 in several autoimmune diseases. Aim of the work: to measure the serum level of sCTLA-4 in patients with immune cytopenia (AIHA, EVANS, ALPS, AND ITP). Patients and methods: forty-seven children and adolescents with immune cytopenias were recruited and assessed for eligibility at the pediatric hematology/ oncology clinic, Ain Shams University children’s hospital and compared with age and gender matched 47 healthy subjects. Results: immune-cytopenic patients group have statistically significant (P<0.01) higher serum sCTLA-4 levels median (IQR) 14 (10 – 20) compared to controls median (IQR) 3 (2 - 6). On the other hand, no significant difference was found as regards levels of sCTLA-4 between sera of ITP, AIHA and ALPS patients (P >0.05). Serum sCTLA-4 was inversely related to the age at diagnosis and positively related to disease duration. Our results demonstrated the presence of negative correlation between sCTLA-4 and current laboratory hemoglobin levels (P= 0.031) and positive correlation to the current reticulocyte count (P= 0.004), indirect bilirubin (P= 0.003) and lactate dehydrogenase (P= 0.014). Conclusion: soluble form of ctla4 (sCTLA-4) presents in elevated levels in the sera of children and adolescents who have immune cytopenia including AIHA/EVANS, ITP and ALPS compared to healthy control group which emphasis its role in the pathogenesis of immune cytopenia. |