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Autoimmune Lymphoproliferative Syndrome (ALPS) in a Boy with Massive Lymphadenopathy

Author(s): Hamid Reza Kianifar | Maryam Khalesi | Reza Farid | Zahra Badiee | Maryam Rastin | Hamid Ahanchian

Journal: Iranian Journal Of Allergy, Asthma and Immunology
ISSN 1735-1502

Volume: 9;
Issue: 3;
Start page: 181;
Date: 2010;
Original page

Keywords: Autoimmune Lymphoproliferative Syndrome | Apoptosis | Cytopenia

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.
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