[Advances in the knowledge and management of autoimmune lymphoproliferative syndrome].
Autoimmune lymphoproliferative syndrome (ALPS) represents a failure of apoptotic mechanisms to maintain lymphocyte homeostasis. ALPS often manifest in childhood with cytopenias, chronic non-malignant lymphoproliferation and autoimmune complications. A number of new insights have improved the understanding of the genetics and biology of ALPS. The treatment of the disease has changed and mycophenolate mofetil and sirolimus have been demonstrated to have marked activity against the disease, improving quality of life for many patients. These will be discussed in this review.
Sección Onco-Hematología Pediátrica, Hospital General Universitario Gregorio Marañón, Madrid, España. Electronic address: cgarridoc@salud.madrid.org.
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Garrido Colino
D056735:Autoimmune Lymphoproliferative Syndrome / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology / Q000628:therapy; D002648:Child; D006801:Humans
Autoimmune lymphoproliferative syndrome; Chronic non-malignant lymphadenopathy; Citopenias recurrentes; Cytopenias; FAS; Gen FAS; Linfadenopatías crónicas; Síndrome linfoproliferativo autoinmune