Tehran University of Medical Sciences Iranian Journal of Allergy, Asthma and Immunology 1735-1502 9 3 2010 09 15 181 183 Hamid Reza Kianifar Department of Pediatric Gastroenterology, Ghaem Medical Center, Mashhad University of Medical Sciences, Mashhad, Iran Maryam Khalesi Department of Pediatric Gastroenterology, Ghaem Medical Center, Mashhad University of Medical Sciences, Mashhad, Iran Reza Farid Department of Immunology, Ghaem Medical Center, Mashhad University of Medical Sciences, Mashhad, Iran. Zahra Badiee Department of Pediatric Hematology, Ghaem Medical Center, Mashhad University of Medical Sciences, Mashhad, Iran Maryam Rastin Immunology Research Center, Buali Research Institute, Mashhad University of Medical Sciences, Mashhad, Iran Hamid Ahanchian Department of Pediatric Immunology, Ghaem Medical Center, Mashhad University of Medical Sciences, Mashhad, Iran 2015 10 01 Autoimmune lymphoproliferative syndrome (ALPS) is an uncommon nonmalignant lymphoproliferative disease which is characterized by chronic, persistent or recurrent lymphadenopathy, splenomegaly, hepatomegaly, immune cytopenia, hypergammaglobinemia and increased risk of lymphoma. We report a 2-year old boy with hepatosplenomegaly as first presentation. Petechial and purpuric rashes with massive cervical lymphadenopathies developed 10 months later. In laboratory tests anemia, thrombocytopenia and hypergammaglobinemia were observed. According to flocytometry increased double negative T cells and by apoptosis assay decrease apoptosis of lymphocytes accompanied clinical manifestations, thus diagnosis of ALPS was established. In conclusion; in all patients with massive lymphadenopathy and hepatosplenomegay; especially with cytopenia; ALPS should be considered. https://ijaai.tums.ac.ir/index.php/ijaai/article/view/285 https://ijaai.tums.ac.ir/index.php/ijaai/article/download/285/285