Article
Natural history of autoimmune lymphoproliferative syndrome associated with FAS gene mutations.
Blood - 27 Mar 2014
Price Susan, Shaw Pamela A, Seitz Amy, Joshi Gyan, Davis Joie, Niemela Julie E, Perkins Katie, Hornung Ronald L, Folio Les, Rosenberg Philip S, Puck Jennifer M, Hsu Amy P, Lo Bernice, Pittaluga Stefania, Jaffe Elaine S, Fleisher Thomas A, Rao V Koneti, Lenardo Michael J
Abstract excerpt
Autoimmune lymphoproliferative syndrome (ALPS) presents in childhood with nonmalignant lymphadenopathy and splenomegaly associated with a characteristic expansion of mature CD4 and CD8 negative or double negative T-cell receptor αβ(+) T lymphocytes. Patients often present with chronic multilineage cytopenias due to autoimmune peripheral destruction and/or splenic sequestration of blood cells and have an increased...
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