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Giant Cell Hepatitis with Autoimmune Hemolytic Anemia; a Case Report

Author(s): Mehri Najafi | Vajiheh Modarresi | Kambiz Eftekhari | Fatemeh Mahjoub | Mina Izadyar

Journal: Iranian Journal of Pediatrics
ISSN 2008-2142

Volume: 19;
Issue: 4;
Start page: 421;
Date: 2009;
Original page

Keywords: Giant cell hepatitis | Autoimmunity | Hemolytic anemia | Rituximab

Background: 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. Case Presentation: 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. Conclusion: 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.
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