Vásconez-Muñoz F, Hernandez-Almeida P, Sandoval-Carrión G, Vásconez-Montalvo A, Flores-Santy K

Language: Spanish
References: 12
Page: 68-71
PDF size: 233.22 Kb.

ABSTRACT

Introduction: giant cell hepatitis with autoimmune hemolytic anemia (GCH-AIHA) is a rare and serious condition that usually occurs during the first year of life. There is no specific diagnostic test; it is histologically characterized by a transformation of hepatocytes into giant cells. Case presentation: male patient who at 45 days-old starts with jaundice, pallor, and hepatomegaly. Leukocytosis, normocytic anemia with positive direct Coombs, and elevation of bilirubin, lactate dehydrogenase and transaminases were found. Serology for syphilis, toxoplasma, rubella, cytomegalovirus, and herpes simplex were negative. Diagnosis was established by histopathological findings in liver biopsy, plus hemolytic anemia. The patient was treated with steroids and azathioprine, and has maintained a satisfactory evolution until the age of eight years, without relapses, and improvement of the histopathological pattern. Conclusions: GCH-AIHA is a serious disease, which should be suspected in infants with hemolytic anemia, plus evidence of acute hepatitis, after ruling out the main causes of hepatitis.

REFERENCES

1. Bernard O, Hadchouel M, Scotto J, Odièvre M, Alagille D. Severe giant cell hepatitis with autoimmune hemolytic anemia in early childhood. J Pediatr. 1981; 99(5): 704-711.

2. Kim YH, Kim JW, Lee EJ, Kang GH, Kang HJ, Moon JS et al. Successful Treatment of a Korean Infant with Giant Cell Hepatitis with Autoimmune Hemolytic Anemia Using Rituximab. Pediatr Gastroenterol Hepatol Nutr. 2020; 23(2): 180-187.

3. Nastasio S, Matarazzo L, Sciveres M, Maggiore G. Giant cell hepatitis associated with autoimmune hemolytic anemia: an update. Transl Gastroenterol Hepatol. 2021; 6: 25.

4. Whitington PF, Vos MB, Bass LM, Melin-Aldana H, Romero R, Roy CC et al. Humoral immune mechanism of liver injury in giant cell hepatitis with autoimmune hemolytic anemia. J Pediatr Gastroenterol Nutr. 2014; 58(1): 74-80.

5. Cho MH, Park HS, Han HS, Bae SH. Giant cell hepatitis with autoimmune hemolytic anemia in a Korean infant. Pediatr Int. 2016; 58(7): 628-631.

6. Poddighe D, Madiyeva A, Talipova D, Umirbekova B. Infantile giant cell hepatitis with autoimmune hemolytic anemia. World J Hepatol. 2021; 13(4): 411-420.

7. Nastasio S, Sciveres M, Maggiore G. Should Giant Cell Hepatitis With Autoimmune Hemolytic Anemia Be Considered a Pediatric Autoimmune Liver Disease? J Pediatr Gastroenterol Nutr. 2018; 66(5): e137.

8. Maggiore G, Sciveres M, Fabre M, Gori L, Pacifico L, Resti M et al. Giant cell hepatitis with autoimmune hemolytic anemia in early childhood: long-term outcome in 16 children. J Pediatr. 2011; 159(1): 127-132.e1.

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

10. Marsalli G, Nastasio S, Sciveres M, Calvo PL, Ramenghi U, Gatti S et al. Efficacy of intravenous immunoglobulin therapy in giant cell hepatitis with autoimmune hemolytic anemia: A multicenter study. Clin Res Hepatol Gastroenterol. 2016; 40(1): 83-89.

11. Matarazzo L, Di Chio T, Nastasio S, Tommasini A, Ventura A, Maggiore G. B-cell depletion induces prolonged remission in patients with giant cell hepatitis and autoimmune hemolytic anemia. Clin Res Hepatol Gastroenterol. 2020; 44(1): 66-72.

12. Bakula A, Socha P, Klaudel-Dreszler M, Karolczyk G, Wozniak M, Rutynowska-Pronicka O et al. Giant cell hepatitis with autoimmune hemolytic anemia in children: Proposal for therapeutic approach. J Pediatr Gastroenterol Nutr. 2014; 58(5): 669-673.