{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,2,21]],"date-time":"2025-02-21T23:16:22Z","timestamp":1740179782420,"version":"3.37.3"},"reference-count":0,"publisher":"Colegio Mexicano de Inmunologia Clinica y Alergia, A. C.","issue":"4","license":[{"start":{"date-parts":[[2019,11,14]],"date-time":"2019-11-14T00:00:00Z","timestamp":1573689600000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["RAM"],"abstract":"<jats:p>El s\u00edndrome linfoproliferativo autoinmune (ALPS, autoimmune lymphoproliferative syndrome) es un error innato de la inmunidad, resultado de un grupo heterog\u00e9neo de alteraciones en los genes que regulan el fen\u00f3meno de apoptosis. Se manifiesta t\u00edpicamente en los primeros a\u00f1os de vida. Las manifestaciones cl\u00ednicas m\u00e1s comunes son la expansi\u00f3n linfoide con linfadenopat\u00eda, esplenomegalia y hepatomegalia, enfermedad autoinmune con citopenias, incluyendo trombocitopenia y anemia hemol\u00edtica, as\u00ed como linfoma. Las anomal\u00edas de laboratorio que facilitan el diagn\u00f3stico de ALPS incluyen presencia de c\u00e9lulas alfa-beta T doble negativas, niveles elevados de interleucina 10, vitamina B12 en sangre y apoptosis defectuosa mediada por FAS en ensayo in vitro. El tratamiento de ALPS se centra en tres aspectos: el tratamiento de las manifestaciones de la enfermedad, la prevenci\u00f3n y tratamiento de las complicaciones y el tratamiento curativo (trasplante de c\u00e9lulas progenitoras hematopoy\u00e9ticas [TCPH]). Se sugiere el uso de tratamiento inmunosupresor solo para las complicaciones graves de la linfoproliferaci\u00f3n o manifestaciones autoinmunes concomitantes. La esplenectom\u00eda no se recomienda para las manifestaciones autoinmunes en pacientes con ALPS. El TCPH se reserva para pacientes seleccionados. La tasa de supervivencia a 50 a\u00f1os se estima en 85\u00a0% para los pacientes con deficiencia de FAS.<\/jats:p>","DOI":"10.29262\/ram.v66i4.646","type":"journal-article","created":{"date-parts":[[2020,2,10]],"date-time":"2020-02-10T04:52:45Z","timestamp":1581310365000},"page":"456-473","source":"Crossref","is-referenced-by-count":0,"title":["S\u00edndrome linfoproliferativo autoinmune.  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