Iranian Journal of Pediatrics
Tehran University of Medical Sciences Press
Vol. 19, No. 4, 2009, pp. 421-424
Bioline Code: pe09053
Full paper language: English
Document type: Case Report
Document available free of charge
Iranian Journal of Pediatrics, Vol. 19, No. 4, 2009, pp. 421-424
© © 2009 by Center of Excellence for Pediatrics, Children’s Medical Center, Tehran University of Medical Sciences,All rights reserved.
Giant Cell Hepatitis with Autoimmune Hemolytic Anemia; a Case Report|
Najafi, Mehri; Modarresi, Vajiheh; Eftekhari, Kambiz; Mahjoub, Fatemeh & Izadyar, Mina
Giant cell hepatitis (GCH) is a histological finding in infants with neonatal cholestasis and rarely can be seen after this period. Autoimmune hemolytic anemia (AIHA) which is characterized by massive and acute red blood cell destruction due to antibody production, responds favorably to corticosteroid therapy. The combination of GCH and AIHA is a rare distinct entity that carries poor response to immunosuppressive therapy and often progresses to fatal liver disease.
A 13-month-old boy was referred to us with anemia and icterus. Investigations confirmed the diagnosis of GCH which was associated with AIHA. Treatment with steroids and azathioprin failed. We changed to cyclosporine. The anemia improved by steroid and IVIG at the beginning, but few months later anemia relapsed. Finally he recovered with rituximab and now he is 6 years old in suitable condition.
The association of AIHA with GCH is an uncommon condition that can be life threatening. Most patients initially respond to immunosuppressive agents, but usually recur and have an aggressive course. In this case liver disease responded to steroid and immunosuppressive therapy, but the AIHA was refractory to immunosuppressive therapy. Rituximab is used in treatment of this condition, and this treatment was well tolerated and resulted in dramatic sustained improvement of anemia.
Giant cell hepatitis; Autoimmunity; Hemolytic anemia; Rituximab
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