Infantile giant cell hepatitis with autoimmune hemolytic anemia

Abstract

Giant cell hepatitis (GCH) is characterized by large and multinucleated (syncytial) hepatocytes in the context of liver inflammation. Infantile GCH is typically associated with autoimmune hemolytic anemia in the absence of any other systemic or organ-specific autoimmune comorbidity. The etiology is unknown; concomitant viral infections (as potential trigger factors) have been identified in a few patients. The pathogenesis reportedly relies upon immune-mediated/ autoimmune mechanisms. This condition should be considered in any infant developing Coombs-positive anemia; indeed, anemia usually precedes the development of hepatitis. The clinical course is usually aggressive without the appropriate immunosuppressive therapy, which may include steroids, conventional immunosuppressors ( e.g. , azathioprine and cyclophosphamide as first-line treatments), intravenous immunoglobulin, and biologics (rituximab). Improvements in medical management (including the availability of rituximab) have significantly reduced the mortality of this condition in the last decade.

Changes in bone marrow and peripheral blood lymphocyte subset findings with onset of hepatitis-associated aplastic anemia

“…Infantile GCH is typically associated with autoimmune hemolytic anemia. [16,17] In our case, the lack of hemolytic anemia in the initial laboratory findings was sufficient to eliminate GCH. In addition, the bone marrow findings ruled out other diagnoses, such as hemophagocytic syndrome, megaloblastic anemia, bone marrow infiltration of malignancy, myelodysplastic syndrome, acute leukemia, and thymoma.…”

“…GCH is characterized by large and multinucleated hepatocytes in the context of liver inflammation. Infantile GCH is typically associated with autoimmune hemolytic anemia [16,17] . In our case, the lack of hemolytic anemia in the initial laboratory findings was sufficient to eliminate GCH.…”

Hepatitis de células gigantes con anemia hemolítica autoinmune

“…Se caracteriza por la asociación de una lesión hepática aguda con transformación difusa de los hepatocitos en células gigantes, más anemia hemolítica. 6 Su fisiopatología se desconoce, aunque se ha propuesto que sea autoinmune, basado en su asociación con otras enfermedades autoinmunes y buena respuesta al tratamiento inmunosupresor. 7 En cinco de los 16 pacientes reportados por Maggiore y colaboradores se documentaron antecedentes familiares de autoinmunidad; 8 sin embargo, en nuestro paciente no hubo este antecedente.…”

“…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. 12 Las principales causas de mortalidad son insuficiencia hepática, complicaciones del trasplante de hígado y sepsis.…”

Enterovirus 71-Induced Autoimmune Hemolytic Anemia in a Boy

“…The cause of AIHA in most cases is idiopathic. However, in a small proportion of patients, AIHA can be the consequence of autoimmune disorders (eg, systemic lupus erythematosus, 2 infantile giant cell hepatitis 3 ), malignancies 4 (eg, lymphoma, 5 solid tumors 6 ) or infections. Infection contributes around 10% of cases.…”