L’epatite gigantocellulare associata ad anemia emolitica autoimmune

Silvia Nastasio (1), Marco Sciveres (2), Giulia Marsalli (1), Giuseppe Maggiore (1)

(1) Dipartimento di Medicina Clinica e Sperimentale, Università di Pisa, Unità di Gastroenterologia Pediatrica Dipartimento Materno-Infantile della Azienda Ospedaliera Universitaria Pisana; (2) Epatologia Pediatrica e Trapianto di fegato pediatrico, ISMETT-UPMC di Palermo

A cura di Francesco Cirillo

Giant cell hepatitis with autoimmune haemolytic anaemia (GcH-aHa) is a rare and aggressive disease of infancy and may have fatal prognosis. it is presumed to be an autoimmune disease, but the mechanism of liver injury is unknown. immunosuppressive therapy may results in remission in mild forms while in severe forms, B cell depletion with rituximab can be an option. Liver transplantation may be a rescue treatment but relapse can occur in the graft.

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