Title of article :
Autoimmune Lymphoproliferative Syndrome (ALPS) in a Boy with Massive Lymphadenopathy
Author/Authors :
Kianifar, Hamid Reza Department of Pediatric Gastroenterology - Ghaem Medical Center - Mashhad University of Medical Sciences , Khalesi, Maryam Department of Pediatric Gastroenterology - Ghaem Medical Center - Mashhad University of Medical Sciences , Farid, Reza Department of Immunology - Ghaem Medical Center - Mashhad University of Medical Science , Badiee, Zahra Department of Pediatric Hematology - Ghaem Medical Center - Mashhad University of Medical Sciences , Rastin, Maryam Immunology Research Center - Buali Research Institute - Mashhad University of Medical Sciences , Ahanchian, Hamid Department of Pediatric Immunology - Ghaem Medical Center - Mashhad University of Medical Sciences
Abstract :
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.
Keywords :
Autoimmune Lymphoproliferative Syndrome , Apoptosis , Cytopenia
Journal title :
Astroparticle Physics
