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Vol. 20. Issue 6.
Pages 341-344 (June - July 2024)
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Vol. 20. Issue 6.
Pages 341-344 (June - July 2024)
Case Report
VEXAS syndrome: A 2-case series report
Síndrome de VEXAS: a propósito de una serie de 2 casos
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Adrián Mayo-Juanateya,
Corresponding author
adrianmayoju@gmail.com

Corresponding author.
, María José Fernández-Llavadorb, María del Mar Fernández-Garcésc, Elia Valls-Pascuala, Juan José Alegre-Sanchoa
a Servicio de Reumatología, Hospital Universitari Doctor Peset, Valencia, Spain
b Servicio de Hematología y Hemoterapia, Hospital Universitari Doctor Peset, Valencia, Spain
c Servicio de Medicina Interna, Hospital Universitari Doctor Peset, Valencia, Spain
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Table 1. Description of clinical cases of VEXAS syndrome diagnosed in our hospital.
Abstract

VEXAS syndrome is a rare entity secondary to UBA1 gene mutations, located on the X chromosome. This mutation generates, as a consequence, a characteristic vacuolation on haematopoietic stem-cells. It is characterized by multiple autoinflammatory and haematologic manifestations, which respond and end up being dependent on corticosteroid treatment. In this publication we present a 2-case series diagnosed at our hospital and make a brief literature review of the published evidence so far.

Keywords:
VEXAS syndrome
Sweet syndrome
Chondritis
Myelodysplastic syndrome
Vasculitis
Resumen

El síndrome VEXAS es una entidad infrecuente secundaria a una mutación en el gen UBA1, localizado en el cromosoma X. Esto provoca como consecuencia la aparición de vacuolas, característica de esta patología, en células madre hematopoyéticas. Se caracteriza por la aparición de múltiples manifestaciones autoinflamatorias sistémicas y hematológicas, que responden y acaban siendo dependientes al tratamiento con glucocorticoides. En la presente publicación aportamos una serie de 2 casos diagnosticados en nuestro centro, y realizamos una breve revisión de la evidencia publicada al respecto.

Palabras clave:
Síndrome de VEXAS
Síndrome de Sweet
Condritis
Síndrome mielodisplásico
Vasculitis

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