Autoimmune Lymphoproliferative Syndrome in Children with Nonmalignant Organomegaly, Chronic Immune Cytopenia, and Newly Diagnosed Lymphoma
This study investigated the frequency of and predictive factors for autoimmune lymphoproliferative syndrome (ALPS) in children with lymphoma, chronic immune cytopenia, and nonmalignant organomegaly. Thirty-four children with suspected ALPS (n=13, lymphoma; n=12, immune cytopenia; n=9, nonmalignant o...
| Main Authors: | , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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Galenos Publishing House
2021-06-01
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| Series: | Turkish Journal of Hematology |
| Subjects: | |
| Online Access: | https://jag.journalagent.com/z4/download_fulltext.asp?pdir=tjh&un=TJH-41514 |
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| author | Zühre Kaya Melek Işık Nihan Oruklu Serap Kirkiz Emin Ümit Bağrıaçık Luis M. Allende María J. Díaz-madroñero Raquel Ruiz-García Faruk Güçlü Pınarlı Pınar Göçün Uyar Ülker Koçak |
| author_facet | Zühre Kaya Melek Işık Nihan Oruklu Serap Kirkiz Emin Ümit Bağrıaçık Luis M. Allende María J. Díaz-madroñero Raquel Ruiz-García Faruk Güçlü Pınarlı Pınar Göçün Uyar Ülker Koçak |
| author_sort | Zühre Kaya |
| collection | DOAJ |
| description | This study investigated the frequency of and predictive factors for autoimmune lymphoproliferative syndrome (ALPS) in children with lymphoma, chronic immune cytopenia, and nonmalignant organomegaly. Thirty-four children with suspected ALPS (n=13, lymphoma; n=12, immune cytopenia; n=9, nonmalignant organomegaly) were included. Double-negative T-cells, lymphocyte apoptosis, and genetic findings were analyzed. Patients were stratified into two groups as proven/probable ALPS and clinically suspected patients according to the ALPS diagnostic criteria. Of the 34 patients, 18 (53%) were diagnosed with proven/probable ALPS. One patient had a mutation (c.652-2A>C) in the FAS gene. The remaining 16 (47%) patients were defined as clinically suspected patients. Predictive factors for ALPS were anemia and thrombocytopenia in patients with lymphoma, splenomegaly and lymphadenopathy in patients with immune cytopenia, and young age in patients with nonmalignant organomegaly. ALPS may not be rare in certain risk groups. Our study indicates that screening for ALPS may be useful in children having lymphoma with cytopenia at diagnosis, in those having nonmalignant organomegaly with immune cytopenia, and in those having chronic immune thrombocytopenic purpura or autoimmune hemolytic anemia with organomegaly developing during follow-up. |
| first_indexed | 2024-04-10T11:54:24Z |
| format | Article |
| id | doaj.art-fe0b60ef578d4432ba9a9b45506dbc8b |
| institution | Directory Open Access Journal |
| issn | 1308-5263 |
| language | English |
| last_indexed | 2024-04-10T11:54:24Z |
| publishDate | 2021-06-01 |
| publisher | Galenos Publishing House |
| record_format | Article |
| series | Turkish Journal of Hematology |
| spelling | doaj.art-fe0b60ef578d4432ba9a9b45506dbc8b2023-02-15T16:16:54ZengGalenos Publishing HouseTurkish Journal of Hematology1308-52632021-06-0138214515010.4274/tjh.galenos.2020.2020.0618TJH-41514Autoimmune Lymphoproliferative Syndrome in Children with Nonmalignant Organomegaly, Chronic Immune Cytopenia, and Newly Diagnosed LymphomaZühre Kaya0Melek Işık1Nihan Oruklu2Serap Kirkiz3Emin Ümit Bağrıaçık4Luis M. Allende5María J. Díaz-madroñero6Raquel Ruiz-García7Faruk Güçlü Pınarlı8Pınar Göçün Uyar9Ülker Koçak10Gazi University Faculty of Medicine, Department of Pediatric Hematology, Ankara, TurkeyGazi University Faculty of Medicine, Department of Pediatric Hematology, Ankara, TurkeyGazi University Faculty of Medicine, Department of Immunology and Life Science Research Center, Ankara, TurkeyGazi University Faculty of Medicine, Department of Pediatric Hematology, Ankara, TurkeyGazi University Faculty of Medicine, Department of Immunology and Life Science Research Center, Ankara, TurkeyImmunology Department and Research Institute i+12, Hospital Universitario 12 de Octubre, Madrid, SpainImmunology Department and Research Institute i+12, Hospital Universitario 12 de Octubre, Madrid, SpainImmunology Department and Research Institute i+12, Hospital Universitario 12 de Octubre, Madrid, SpainGazi University Faculty of Medicine, Department of Pediatric Oncology, Ankara, TurkeyGazi University Faculty of Medicine, Department of Pathology, Ankara, TurkeyGazi University Faculty of Medicine, Department of Pediatric Hematology, Ankara, TurkeyThis study investigated the frequency of and predictive factors for autoimmune lymphoproliferative syndrome (ALPS) in children with lymphoma, chronic immune cytopenia, and nonmalignant organomegaly. Thirty-four children with suspected ALPS (n=13, lymphoma; n=12, immune cytopenia; n=9, nonmalignant organomegaly) were included. Double-negative T-cells, lymphocyte apoptosis, and genetic findings were analyzed. Patients were stratified into two groups as proven/probable ALPS and clinically suspected patients according to the ALPS diagnostic criteria. Of the 34 patients, 18 (53%) were diagnosed with proven/probable ALPS. One patient had a mutation (c.652-2A>C) in the FAS gene. The remaining 16 (47%) patients were defined as clinically suspected patients. Predictive factors for ALPS were anemia and thrombocytopenia in patients with lymphoma, splenomegaly and lymphadenopathy in patients with immune cytopenia, and young age in patients with nonmalignant organomegaly. ALPS may not be rare in certain risk groups. Our study indicates that screening for ALPS may be useful in children having lymphoma with cytopenia at diagnosis, in those having nonmalignant organomegaly with immune cytopenia, and in those having chronic immune thrombocytopenic purpura or autoimmune hemolytic anemia with organomegaly developing during follow-up.https://jag.journalagent.com/z4/download_fulltext.asp?pdir=tjh&un=TJH-41514autoimmune lymphoproliferative syndromeimmune cytopenialymphoma |
| spellingShingle | Zühre Kaya Melek Işık Nihan Oruklu Serap Kirkiz Emin Ümit Bağrıaçık Luis M. Allende María J. Díaz-madroñero Raquel Ruiz-García Faruk Güçlü Pınarlı Pınar Göçün Uyar Ülker Koçak Autoimmune Lymphoproliferative Syndrome in Children with Nonmalignant Organomegaly, Chronic Immune Cytopenia, and Newly Diagnosed Lymphoma Turkish Journal of Hematology autoimmune lymphoproliferative syndrome immune cytopenia lymphoma |
| title | Autoimmune Lymphoproliferative Syndrome in Children with Nonmalignant Organomegaly, Chronic Immune Cytopenia, and Newly Diagnosed Lymphoma |
| title_full | Autoimmune Lymphoproliferative Syndrome in Children with Nonmalignant Organomegaly, Chronic Immune Cytopenia, and Newly Diagnosed Lymphoma |
| title_fullStr | Autoimmune Lymphoproliferative Syndrome in Children with Nonmalignant Organomegaly, Chronic Immune Cytopenia, and Newly Diagnosed Lymphoma |
| title_full_unstemmed | Autoimmune Lymphoproliferative Syndrome in Children with Nonmalignant Organomegaly, Chronic Immune Cytopenia, and Newly Diagnosed Lymphoma |
| title_short | Autoimmune Lymphoproliferative Syndrome in Children with Nonmalignant Organomegaly, Chronic Immune Cytopenia, and Newly Diagnosed Lymphoma |
| title_sort | autoimmune lymphoproliferative syndrome in children with nonmalignant organomegaly chronic immune cytopenia and newly diagnosed lymphoma |
| topic | autoimmune lymphoproliferative syndrome immune cytopenia lymphoma |
| url | https://jag.journalagent.com/z4/download_fulltext.asp?pdir=tjh&un=TJH-41514 |
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