Giant Cell Hepatitis With Autoimmune Hemolytic Anemia in Children

Abstract

We propose Rtx as the treatment of choice for severe GCH with AIHA in the early stages of the disease, provided steroids and azathioprine are ineffective.

Giant cell hepatitis with autoimmune hemolytic anemia in a Korean infant

“…suggested that systemic B‐lymphocyte autoimmunity plays a central role in the pathologic mechanism . In contrast to autoimmune hepatitis, however, autoantibodies associated with liver disease, such as ANA, anti‐smooth muscle antibody, anti‐mitochondrial antibody, and anti‐liver–kidney microsomal antibody, were found in a few cases . In the present case, ANA and anti‐smooth muscle antibodies were found at initial diagnosis.…”

Efficacy of intravenous immunoglobulin therapy in giant cell hepatitis with autoimmune hemolytic anemia: A multicenter study

“…Rituximab (RTX), which is believed, in particular, to deplete CD20-positive autoreactive plasma cells sparing protective antimicrobial plasma cells [18], was recently shown to induce and maintain remission in a small series of patients with severe liver disease [9,10]. Moreover, RTX has been also shown to act as a steroid-sparing drug, permitting patients to be weaned off corticosteroid therapy, particularly in case of an early treatment [10].…”

“…A recent study demonstrated a diffuse C5b-9 complex deposition on the membrane of giant hepatocytes, suggesting that liver cell injury might be autoantibodydriven and complement-mediated, as in the case of the associated Coombs-positive hemolytic anaemia [8]. Moreover prolonged remission has been recently reported using B cell depletion immunotherapy with chimeric anti-CD20 monoclonal antibody (rituximab) [2,9,10].…”

“…Intravenous immunoglobulin (IVIg) has been empirically used in patients with GCH-AHA, mainly at replacement dose and in single administration, for treating the autoimmune hemolytic anemia before the onset of liver disease, but without proof of efficacy [3,6,9,11,12]. In a few patients, IVIg has been used, in association with cyclosporine [6], plasmapheresis [9,14], rituximab [15] for the treatment of the liver disease.…”

“…In a few patients, IVIg has been used, in association with cyclosporine [6], plasmapheresis [9,14], rituximab [15] for the treatment of the liver disease. Results, however, were not conclusive and usually limited to a slight reduction of aminotransferase activity.…”