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Abstract Summary utoimmune cytopenias are characterized by the production of autoantibodies against differentiated hematopoietic cells because of defects in central and/or peripheral tolerance. It includes autoimmune hemolytic anemia (AIHA), immune thrombocytopenic purpura (ITP), autoimmune proliferative syndrome (ALPS) and Evans syndrome (EV). Cytotoxic T lymphocyte associated antigen-4 (CTLA-4) is a costimulatory receptor that has a potent inhibitory signal on antigen-activated immune responses and was detected in many autoimmune disorders. A native soluble form of CTLA-4 (sCTLA-4) has been found to interfere with normal immune response leading to auto-reactivity. It was observed in high levels in different autoimmune diseases such as systemic lupus erythematosus, autoimmune thyroid disease, celiac disease and systemic sclerosis. The association between levels of sCTLA-4 and autoimmune cytopenias of children and adolescents patients such as AIHA, ITP, ALPS and ES has not investigated through literature. The aims of our study were to compare levels of soluble CTLA-4 in different types of immune cytopenias and their control in children and adolescent patients and to determine the possible relation between sCTLA-4 and the disease severity and response to treatment in this group of patients. A Summary 84 Forty seven children and adolescents who have autoimmune cytopenias were recruited and assessed for eligibility in Pediatric Hematology Clinic, Ain Shams University Children‘s Hospital and forming a patients group with age range 8 – 204 months old. An age and sex matched healthy control group were recruited including forty seven healthy participants with age range 6 – 156 months old. Both patients and control groups were subjected to full history taking, general examination and laboratory evaluation. We also obtained the initial laboratory examinations and the treatment protocols of the patients groups. Our results showed upon comparison of patient group to control group that patient group have significantly higher levels of sCTLA4 and no significant differences between the ITP, AIHA and ALPS patients regarding the levels of measured sCTLA-4. Our results revealed that there was no correlation between the age at the study entry of the autoimmune cytopenic patients and levels of sCTLA-4 (R= -0.028, P= 0.853). Our results showed that sCTLA-4 is inversely related to the platelet count (R= -0.324, P<0.05) and moreover, there was a highly significant negative correlations between level of sCTLA-4 and both hepatosplenomegaly and recurrent infections and significant correlation between level of sCTLA-4 and treatment protocol (steroids+ steroid sparing). Summary 85 There was a highly significant differences in initial and current laboratory platelets level among ITP, AIHA and ALPS patients (P <0.05). Its level was the highest among AIHA patients and ALPS patients in compare with ITP. Our findings revealed a significant differences in the prevalence of hepatosplenomegaly among ITP, AIHA and ALPS patients (P <0.05) with higher prevalence among ALPS patients. There was a significant difference in the prevalence of cardiac manifestations among autoimmune cytopenias patients with a higher prevalence among AIHA patients in compare with ITP and ALPS patients. Our results revealed that the rate of recurrent infection among ALPS and AIHA patients were high in comparing with ITP patient |