Giant cell hepatitis with autoimmune hemolytic anemia in a Korean infant

Abstract

Giant cell hepatitis (GCH) with autoimmune hemolytic anemia (AHA) is a very rare disease characterized by early onset and severe clinical manifestations, including immune hemolytic anemia and hepatitis with cholestasis. The prognosis is poor despite aggressive immunosuppressive therapy. We report here the first case of GCH with AHA in East Asia. A 2-month-old boy was admitted with jaundice. Blood test indicated abnormal liver function and low hemoglobin. Direct Coombs test and several autoantibodies associated with liver disease were positive, and liver biopsy was consistent with GCH. He was treated with prednisolone and ursodeoxycholic acid, and at the time of writing was in clinical and biochemical remission after prednisolone was stopped.

Reptin/RUVBL2 is required for hepatocyte proliferation in vivo, liver regeneration and homeostasis

“…Interestingly, 36 h after PH, the only 1% of hepatocytes that were BrdU positive displayed an increased size, similar to hypertrophic hepatocytes observed at 4 weeks without PH (Figure 1C). Only few clinical cases of rare giant cell hepatitis described the existence of large cells in the liver after severe liver failure, 16 and one study of Raptor KO in the mouse liver, a critical mTORC1 component, has shown hypertrophic hepatocytes 17 . Hypertrophic cells in Reptin LKO display a hepatocyte morphology and are highly proliferative.…”

Successful Treatment of a Korean Infant with Giant Cell Hepatitis with Autoimmune Hemolytic Anemia Using Rituximab

“…Majority of the cases have been described in Caucasian populations. However, very few cases from East Asia have also been reported [5].…”

Hepatitis de células gigantes con anemia hemolítica autoinmune

“…4 Para el tratamiento, los inmunosupresores (prednisona y azatioprina) proporcionan una adecuada respuesta inicial en la mayoría de los pacientes; no obstante, pueden ocurrir recaídas. 5 Sin embargo, se considera que esta entidad es grave, ya que los pacientes tienen una evolución posiblemente letal a pesar del diagnóstico y tratamiento oportuno; 3 la tasa de mortalidad puede llegar hasta 30%. 2 Presentamos el caso de un niño diagnosticado de HCG-AHAI a los dos meses de edad, quien presentó una adecuada respuesta al tratamiento inmunosupresor.…”