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

2019, Number 3

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Alerg Asma Inmunol Pediatr 2019; 28 (3)

Autoimmunity in Wiskott-Aldrich syndrome

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

Language: Spanish
References: 26
Page: 83-87
PDF size: 207.43 Kb.


ABSTRACT

Wiskott-Aldrich syndrome (WAS) is a primary immunodeficiency linked to chromosome X that is characterized by the classic triad of eczema, severe immunodeficiency and microthrombocytopenia. The defective function in the WAS protein (WASP) affects the cells of the innate and adaptive immune system, which require an adequate functioning of their cellular cytoskeleton It is estimated that around 40% of patients with WAS develop autoimmunity. Chronic inflammation, interleukin-2 deficiency and increased apoptosis seem to be responsible for the loss of peripheral tolerance to the antigens themselves in this disease.


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