ETIOLOGY OF INCREASED TRANSAMINASES IN A PATIENT WITH AUTOIMMUNE
HEMOLYTIC ANEMIA: GIANT CELL HEPATITIS
Burcu Kılınç1, Simge Çınar Özel2, Ayşe Gonca Kaçar1, Erkan Akkuş3, Süheyla Ocak1, Ömer Faruk Beşer3,
Fügen Çullu Çokuğraş3 and Tiraje Celkan1
(1)1. Istanbul University-Cerrahpasa, Cerrahpasa Medical Faculty, Depatment of Pediatric Hematology
and Oncology, Istanbul, Turkey
(2)Istanbul University-Cerrahpaşa Cerrahpaşa Medical Faculty, Pediatric Hematology and Oncology,
Istanbul, Turkey
(3)Istanbul University-Cerrahpaşa Cerrahpaşa Medical Faculty, Pediatric Gastroenterology,
Hepatology, Istanbul, Turkey
Introduction: The association of autoimmune hemolytic anemia and giant cell hepatitis(GCH-AHA)
is a rare autoimmune disease seen in infants and early childhood. It causes severe
damage due to giant cell infiltration in the liver and coombs positive hemolytic anemia(1,2).
Although autoantibodies are negative, coombs positivity and response to immunosuppressive
therapy support that it is an autoimmune disease. We will present a 10-month-old case
diagnosed with GCH-AHA while investigating the etiology of increased transaminases.
Case report: The patient was a 10 month-old boy. He was the first child of unrelated parents.
He was born term and had no complaints until 6 months. Anemia was detected at the age of
6 months one week after the meningococcal vaccine. He was diagnosed with mixed type AHA
because of reticulocytosis and coombs IgG + C +. There was no significant remission with
steroid treatment.The patient received intermittent red cell transfusion and intravenous
immunoglobulin(IVIG). Steroid treatment was tapered and cyclosporine was started. The
patient was followed up in remission for 2 months with cyclosporine. At 4th month of the
diagnosis, severe transaminase elevation (serum aspartate aminotransferase(AST): 4841 IU /
L, serum alanine aminotransferase (ALT): 3988 IU / L), hyperbilirubinemia (Total bilirubin: 20.1
mg / dl), jaundice and hepatomegaly were observed. The patient was evaluated for viral, toxic
and autoimmune hepatitis. Viral tests were normal, autoimmune markers were negative. All
medications of the patient were discontinued and evaluated, but there was no decrease in
transaminases. Liver biopsy was performed. Biopsy revealed giant cell hepatitis and diffuse
fibrosis in the parenchyma. Steroid was started again and cyclosporine was stopped. There
was no significant regression in transaminases with steroids and then rituximab treatment
was started. A total of four doses rituximab was given 375 mg / m2 once a week . His blood
test results at 15 days after the end of the treatment were hemoglobin:11.6 gr / dl,
reticulocyte percent: 2.4%, ALT: 212 IU / L, AST: 121 IU / L, Bilirubin: 1.4 mg / dl. Hepatomegaly
and jaundice were regressed. The patient is currently being followed up in remission.
Conclusion: GCH-AHA is a rare disease that occurs at an early age. It has a severe course and
may require transplantation due to liver damage. In cases where it does not respond to
standard immunosuppressive treatments, remission can be achieved with rituximab.
Considering giant cell hepatitis is valuable in terms of treatment course when elevated
transaminases are detected under immunosuppressive therapy in patients diagnosed with
mixed-type AIHA at an early age.