Autoimmune Lymphoproliferative Syndrome (ALPS) in a Boy with Massive Lymphadenopathy
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 ol...
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| Format: | Article |
| Language: | English |
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Tehran University of Medical Sciences
2010-09-01
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| Series: | Iranian Journal of Allergy, Asthma and Immunology |
| Subjects: | |
| Online Access: | https://ijaai.tums.ac.ir/index.php/ijaai/article/view/285 |
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| author | Hamid Reza Kianifar Maryam Khalesi Reza Farid Zahra Badiee Maryam Rastin Hamid Ahanchian |
| author_facet | Hamid Reza Kianifar Maryam Khalesi Reza Farid Zahra Badiee Maryam Rastin Hamid Ahanchian |
| author_sort | Hamid Reza Kianifar |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-04-12T20:08:13Z |
| format | Article |
| id | doaj.art-908977f7bd8d47a299d000000b07f786 |
| institution | Directory Open Access Journal |
| issn | 1735-1502 1735-5249 |
| language | English |
| last_indexed | 2024-04-12T20:08:13Z |
| publishDate | 2010-09-01 |
| publisher | Tehran University of Medical Sciences |
| record_format | Article |
| series | Iranian Journal of Allergy, Asthma and Immunology |
| spelling | doaj.art-908977f7bd8d47a299d000000b07f7862022-12-22T03:18:19ZengTehran University of Medical SciencesIranian Journal of Allergy, Asthma and Immunology1735-15021735-52492010-09-0193285Autoimmune Lymphoproliferative Syndrome (ALPS) in a Boy with Massive LymphadenopathyHamid Reza Kianifar0Maryam Khalesi1Reza Farid2Zahra Badiee3Maryam Rastin4Hamid Ahanchian5Department of Pediatric Gastroenterology, Ghaem Medical Center, Mashhad University of Medical Sciences, Mashhad, IranDepartment of Pediatric Gastroenterology, Ghaem Medical Center, Mashhad University of Medical Sciences, Mashhad, IranDepartment of Immunology, Ghaem Medical Center, Mashhad University of Medical Sciences, Mashhad, Iran.Department of Pediatric Hematology, Ghaem Medical Center, Mashhad University of Medical Sciences, Mashhad, IranImmunology Research Center, Buali Research Institute, Mashhad University of Medical Sciences, Mashhad, IranDepartment of Pediatric Immunology, Ghaem Medical Center, Mashhad University of Medical Sciences, Mashhad, IranAutoimmune 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/285Autoimmune Lymphoproliferative SyndromeApoptosisCytopenia |
| spellingShingle | Hamid Reza Kianifar Maryam Khalesi Reza Farid Zahra Badiee Maryam Rastin Hamid Ahanchian Autoimmune Lymphoproliferative Syndrome (ALPS) in a Boy with Massive Lymphadenopathy Iranian Journal of Allergy, Asthma and Immunology Autoimmune Lymphoproliferative Syndrome Apoptosis Cytopenia |
| title | Autoimmune Lymphoproliferative Syndrome (ALPS) in a Boy with Massive Lymphadenopathy |
| title_full | Autoimmune Lymphoproliferative Syndrome (ALPS) in a Boy with Massive Lymphadenopathy |
| title_fullStr | Autoimmune Lymphoproliferative Syndrome (ALPS) in a Boy with Massive Lymphadenopathy |
| title_full_unstemmed | Autoimmune Lymphoproliferative Syndrome (ALPS) in a Boy with Massive Lymphadenopathy |
| title_short | Autoimmune Lymphoproliferative Syndrome (ALPS) in a Boy with Massive Lymphadenopathy |
| title_sort | autoimmune lymphoproliferative syndrome alps in a boy with massive lymphadenopathy |
| topic | Autoimmune Lymphoproliferative Syndrome Apoptosis Cytopenia |
| url | https://ijaai.tums.ac.ir/index.php/ijaai/article/view/285 |
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