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Órgano Oficial del Colegio Mexicano de Alergia, Asma e Inmunología Pediátrica y de la Asociación Latinoamericana de Pediatría

2019, Número 3

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Alerg Asma Inmunol Pediatr 2019; 28 (3)


Autoinmunidad en el síndrome de Wiskott-Aldrich

Román-Razo EA, González-Serrano E, Espinosa-Padilla S

Idioma: Español
Referencias bibliográficas: 26
Paginas: 83-87
Archivo PDF: 207.43 Kb.


RESUMEN

El síndrome de Wiskott-Aldrich (SWA) es una inmunodeficiencia primaria ligada al cromosoma X que se caracteriza por la tríada clásica de eccema, inmunodeficiencia severa y microtrombocitopenia. La función defectuosa en la proteína WAS (WASP) afecta a las células del sistema inmune innato y adaptativo, que requieren un adecuado funcionamiento del citoesqueleto celular para su funcionamiento. Se estima que alrededor del 40% de los pacientes con SWA desarrolla autoinmunidad. La inflamación crónica, la deficiencia de interleucina-2 y el aumento de la apoptosis parecen ser causantes de la pérdida de la tolerancia periférica a los antígenos propios en esta enfermedad.


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