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Date publication
septembre 2022
Journal
Journal of pediatric gastroenterology and nutrition
Auteurs
Membres identifiés du Cancéropôle Est :
Pr HUET Frédéric
Tous les auteurs :
Gonnot M, Neumann F, Huet F, Maudinas R, Leblanc T, Lacaille F
Lien Pubmed
Résumé
Hepatitis-associated aplastic anemia (HAAA) accounts for 4% of autoimmune hepatitis in children. An episode of seronegative autoimmune hepatitis is followed a few days or months later by aplastic anemia or full aplasia. This autoimmune disease could be due to a regulation defect in the immune response to a viral trigger, in a genetically predisposed individual. Other causes of hepatitis or aplastic anemia have to be ruled out. Steroids and azathioprine usually control the liver damage but do not prevent the development of aplastic anemia. Aplastic anemia is treated with either hematopoietic stem cell transplantation in patients with a sibling donor, or anti-thymocyte globulins and cyclosporine. We propose guidelines to explore and treat this rare disease. We emphasize on the necessary close collaboration between hepatologists and hematologists.
Référence
J Pediatr Gastroenterol Nutr. 2022 09 6;:
