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Evans syndrome is an autoimmune disease in which an individual's antibodies attack their own red blood cells and platelets.
Although Evans syndrome seems to be a disorder of immune regulation, the exact pathophysiology is unknown.
It has been variously reported that between 10% and 23% of patients who have autoimmune hemolytic anemia, will also have thrombocytopenia and thus Evans syndrome.
In approximately one percent of cases, autoimmune hemolytic anemia and ITP coexist, a condition referred to as Evans syndrome.
It has been observed that there is a risk of developing other autoimmune problems and hypogammaglobulinemia, with recent research finding that 58% of children with Evans syndrome have CD4-/CD8- T cells which is a strong predictor for having autoimmune lymphoproliferative syndrome.