International Journal of Academic Health and Medical Research (IJAHMR)   Year: 2023 | Volume: 7 | Issue: 11 | Page No.: 51-53 Autoimmune Lymphoproliferative Syndrome In Children: A Case Report Download PDF S. El moudden, I. JERRAR, S. abourazzak, S. CHAOuki, M.HIDA Abstract: Autoimmune lymphoproliferative syndrome (ALPS), a rare genetic disorder, is a non-malignant, non-infectious lymphoproliferation that alters immune system homeostasis by altering FAS-mediated lymphocyte apoptosis. In the past few years, the diagnosis of ALPS was always confused with other idiopathic autoimmune pathologies such as Evans syndrome (which combines autoimmune hemolytic anemia and idiopathic thrombocytosis), histyocytosis or systemic lupus erythematosus. In this article, we present a case of a child aged 2 years and 7 months, who presented with prolonged fever associated with a tumor syndrome consisting of polyadenopathy, hepatomegaly, splenomegaly and recurrent cytopenia. After eliminating other diagnoses, the diagnosis of autoimmune lymphoproliferative syndrome was retained by the demonstration of the FAS mutation on genetic study.