SÍNDROME DE JOB O ENFERMEDAD ALÉRGICA CON HIPERPRODUCCIÓN DE IGE: A PROPÓSITO DE UN PACIENTE

Vicente José Hernández Moreno, Elena Carvajal Ciomina, Manuela Herrera Martínez

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Resumen

El síndrome de híper IgE (síndrome de Job o de Buckley) es una inmunodeficiencia primaria, rara y compleja, caracterizada por la presencia de altos niveles séricos de IgE (superiores a 2 000 UI/mL3.),eccema crónico, abscesos por infecciones recurrentes de Staphylococcus aureus, infecciones del tracto respiratorio con formación de neumatoceles pulmonares, quimiotaxis neutrofílica reducida,y una serie de características fenotípicas, con anomalías craneofaciales, dentales, músculo-esqueléticas, neurológicas y vasculares, de origen, tanto autosómico dominante como recesivo.

Palabras clave

síndrome de job/diagnóstico.


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