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

Abstract

Giant cell hepatitis with autoimmune hemolytic anemia (AHA) is a rare disease of infancy characterized by the presence of both Coombs-positive hemolytic anemia and progressive liver disease with giant cell transformation of hepatocytes. Here, we report a case involving a seven-month-old male infant who presented with AHA followed by cholestatic hepatitis. The clinical features included jaundice, pallor, and red urine. Physical examination showed generalized icterus and splenomegaly. The laboratory findings suggested warm-type AHA with cholestatic hepatitis. Liver biopsy revealed giant cell transformation of hepatocytes and moderate lobular inflammation. The patient was successfully treated with four doses of rituximab. Early relapse of hemolytic anemia and hepatitis was observed, which prompted the use of an additional salvage dose of rituximab. He is currently in clinical remission.

Óriássejtes hepatitis és autoimmun haemolyticus anaemia társulása kisdedkorban

“…Ritkaságát jelzi, hogy az angol nyelvű világirodalomban 54 esetről található említés [1], tudomásunk szerint az első magyarországi esetet 2012-ben Meczner és mtsai közölték [2]. Bár a GCH-AIHA pontos etiológiája nem ismert, autoimmun mechanizmus valószínűsíthető, B-sejt mediálta immunválasszal [3]. A haemostasiszavar rendezését követően ultrahangvezérelt májbiopszia történt.…”

Hepatitis de células gigantes con anemia hemolítica autoinmune

“…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.…”

“…3,10 Recientemente, parece ser que con el uso de terapia biológica con rituximab la mortalidad ha disminuido de 45 a 10%; 6 con este tratamiento se evitan los efectos adversos del uso prolongado de esteroides, y se consigue remisión completa. 2,11 Se debe considerar que con el tratamiento existe mejoría significativa del componente hematológico, pero el problema hepático se resuelve en alrededor de 50% de los pacientes. 6 Por esta razón, el pronóstico sigue siendo incierto, ya que puede haber falta de respuesta al tratamiento y recaídas.…”

Infantile giant cell hepatitis with autoimmune hemolytic anemia

“…Both males and females can be affected, without a clear gender preponderance; in the largest cases series published by Maggiore et al [ 9 ], there were 9 male and 7 female patients[ 9 ]. If all other case reports and small case series are considered (Table 1 )[ 9 , 11 - 14 , 18 , 22 - 41 ], among the 51 reported patients with infantile GCH + AHA, 25 were female and 19 were male (no gender specification was available for 7 patients).…”

“…immunosuppressive) approach over time. Indeed, if the cases reported after 2011 are specifically considered, only 3 of 29 patients died, which corresponds to an overall mortality rate as low as 10% (Table 1 )[ 9 , 11 - 14 , 18 , 22 - 41 ]. The therapeutic regimens described in these case reports and small case series were widely heterogeneous.…”