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giant cell hepatitis with autoimmune hemolytic anemia

Monday 13 June 2005

Autoimmune hemolytic anemia (AIHA) with giant cell hepatitis (GCH) is an uncommon disease in children and is associated with an aggressive and often fatal course.

Synopsis

- autoimmune hemolytic anemia (AIHA)
- post-infantile giant cell hepatitis

  • diffuse giant cell hepatocyte transformation
  • lobular architecture distortion
  • portal inflammation
  • portal fibrosis
  • lobular pericellular fibrosis
  • micronodular cirrhosis

- hemophagocytosis (11345187)
- Evans syndrome

Treatment

- alemtuzumab (18030239)
- rituximab (17460499)
- cyclophosphamide (16266839)
- liver transplantation (16176422)

See also

- Hepatitis

  • autoimmune hepatitis
  • giant cell hepatitis

References

- Autoimmune hemolytic anemia in an infant with giant cell hepatitis.
Kashyap R, Sarangi JN, Choudhry VP. Am J Hematol. 2006 Mar;81(3):199-201. PMID: 16493610

- Giant cell hepatitis with autoimmune hemolytic anemia and hemophagocytosis. Hartman C, Berkowitz D, Brik R, Arad A, Elhasid R, Shamir R. J Pediatr Gastroenterol Nutr. 2001 Mar;32(3):330-4. PMID: 11345187

- Perez-Atayde AR, Sirlin SM, Jonas M. Coombs-positive autoimmune hemolytic anemia and postinfantile giant cell hepatitis in children. Pediatr Pathol. 1994 Jan-Feb;14(1):69-77. PMID: 8159622

- Bernard O, Hadchouel M, Scotto J, Odievre M, Alagille D. Severe giant cell hepatitis with autoimmune hemolytic anemia in early childhood. J Pediatr. 1981 Nov;99(5):704-11. PMID: 7299542