Contributions
Abstract: EP702
Type: E-Poster Presentation
Session title: Enzymopathies, membranopathies and other anemias
Background
Cytotoxic T lymphocyte associated antigen-4 (CTLA-4) is a costimulatory receptor exhibiting a potent inhibitory signal on antigen-activated immune responses. A soluble form of CTLA-4 (sCTLA-4), resulting from an alternative splicing, has been identified and was found increased in several autoimmune diseases.
Aims
to evaluate serum levels of sCTLA-4 in different types of immune cytopenias and to determine its possible relation to the disease activity.
Methods
Serum levels of sCTLA-4 were assessed using enzyme linked immunosorbent assay in 47 children and adolescents with immune cytopenias and compared to 47 age- and sex-matched healthy controls.
Results
Among the 47 patients enrolled, 24 (51.1%) had chronic immune thrombocytopenia (ITP), 19 (40.4%) had autoimmune hemolytic anemia (AIHA), and 4(8.5%) patients with autoimmune lymphoproliferative disease (ALPS). sCTLA-4 levels were significantly higher in the sera of patients with immune cytopenias as compared to healthy controls (p< 0.001), however, levels were comparable between the different groups of immune cytopenias (p=0.084). Serum sCTLA-4 inversely correlated to the age at diagnosis, and hemoglobin level and (p= 0.048, and p=0.039 respectively), while it directly correlated with the disease duration (p=0.023) as well as markers of hemolysis including reticulocyte count, serum LDH and indirect bilirubin (p=0.025; p=0.019; p=0.004 respectively). Moreover, serum sCTLA-4 levels were significantly lower in patients with AIHA in remission compared to patients with active disease (p=0.026), however, this was not true among the ITP group (p=0.523).
Conclusion
Our results showed that Children with immune cytopenia exhibit significantly higher levels of circulating sCTLA-4 which correlated to the disease activity. The prognostic significance of the assessment of the circulating sCTLA-4 among patients with immune cytopenia and its use to tailor treatment regimen require additional studies.
Keyword(s): Autoimmune hemolytic anemia (AIHA), CTLA-4, Immune thrombocytopenia (ITP)
Abstract: EP702
Type: E-Poster Presentation
Session title: Enzymopathies, membranopathies and other anemias
Background
Cytotoxic T lymphocyte associated antigen-4 (CTLA-4) is a costimulatory receptor exhibiting a potent inhibitory signal on antigen-activated immune responses. A soluble form of CTLA-4 (sCTLA-4), resulting from an alternative splicing, has been identified and was found increased in several autoimmune diseases.
Aims
to evaluate serum levels of sCTLA-4 in different types of immune cytopenias and to determine its possible relation to the disease activity.
Methods
Serum levels of sCTLA-4 were assessed using enzyme linked immunosorbent assay in 47 children and adolescents with immune cytopenias and compared to 47 age- and sex-matched healthy controls.
Results
Among the 47 patients enrolled, 24 (51.1%) had chronic immune thrombocytopenia (ITP), 19 (40.4%) had autoimmune hemolytic anemia (AIHA), and 4(8.5%) patients with autoimmune lymphoproliferative disease (ALPS). sCTLA-4 levels were significantly higher in the sera of patients with immune cytopenias as compared to healthy controls (p< 0.001), however, levels were comparable between the different groups of immune cytopenias (p=0.084). Serum sCTLA-4 inversely correlated to the age at diagnosis, and hemoglobin level and (p= 0.048, and p=0.039 respectively), while it directly correlated with the disease duration (p=0.023) as well as markers of hemolysis including reticulocyte count, serum LDH and indirect bilirubin (p=0.025; p=0.019; p=0.004 respectively). Moreover, serum sCTLA-4 levels were significantly lower in patients with AIHA in remission compared to patients with active disease (p=0.026), however, this was not true among the ITP group (p=0.523).
Conclusion
Our results showed that Children with immune cytopenia exhibit significantly higher levels of circulating sCTLA-4 which correlated to the disease activity. The prognostic significance of the assessment of the circulating sCTLA-4 among patients with immune cytopenia and its use to tailor treatment regimen require additional studies.
Keyword(s): Autoimmune hemolytic anemia (AIHA), CTLA-4, Immune thrombocytopenia (ITP)