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Published in 2021 at "Expert Review of Clinical Immunology"
DOI: 10.1080/1744666x.2021.1978842
Abstract: ABSTRACT Introduction Autoimmune lymphoproliferative syndrome (ALPS) is a rare disorder of immune dysregulation characterized by derangements in first apoptosis signal-mediated apoptosis and elevations in CD3+TCRαβ+CD4−CD8− ‘double negative’ T cells. As our understanding of this pleomorphic…
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Keywords:
diagnosis;
lymphoproliferative syndrome;
presentation diagnosis;
syndrome alps ... See more keywords
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Published in 2017 at "Journal of Pediatric Hematology/Oncology"
DOI: 10.1097/mph.0000000000000785
Abstract: Autoimmune lymphoproliferative syndrome (ALPS) is a disorder of lymphocyte apoptosis. Children present with chronic nonmalignant lymphadenopathy, hepatosplenomegaly, and autoimmune cytopenias. Recent advances show efficacy of treatment with immunosuppressive drugs. Sirolimus, an mammalian target of rapamycin…
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Keywords:
lymphoproliferation;
syndrome alps;
treatment;
lymphoproliferative syndrome ... See more keywords