Hepatitis-Associated Aplastic Anemia in Children: Our 4 Cases

Background: Literature reports just a few cases of Hepatitis- associated aplastic anemia (HAAA) -approximately 200 cases until 1975, with a higher prevalence in adolescent boys. Autoimmunity is thought to be the underlying mechanism, but due to scarce prevalence, little is known.

Objective: To follow and describe cases with autoimmune hepatitis (AIH) and autoimmune associated disorders (AAD), respectively aplastic anemia.

Methods: We conducted a prospective longitudinal study during 1^st of January 2010-1^st of January 2017 in the Paediatric Department of “Grigore Alexandrescu” Children`s Hospital in Bucharest. We included 11 children with AIH-AAD, out of which were 4 cases with aplastic anemia. Diagnosis was established after ruling out viral, metabolic and toxic etiology.

Results: Three of the 4 cases were diagnosed as unclassified hepatitis with negative autoimmune antibodies. The forth case had Wilson disease (WD). HAAA cases were teenagers, 3 boys and a girl aged 16, 14, 14 and 13 years. All of them had been diagnosed with medullary aplasia at 3 to 4 months after an attack of acute hepatitis, with levels of transaminases within the range of 1000 to 3500, with the exception of WD case, with values less than 600 U/L.

We did not perform hepatic biopsy in none of the patients, because at the onset we considered acute hepatitis, probably viral with unidentified infectious agent. The remission of hepatic cytolysis occurred within the first month, so we did not find it necessary afterwards. Two patients received supportive treatment and one immunosuppressive therapy. For 3 cases the outcome was unfavourable, with deceases in short time due to severe pancytopenia. The only survivor in the group was the girl, with complete recovery at 6 years follow-up.

Conclusions: Children with seronegative AIH should be monitored at least 6 months in order to catch the bone marrow failure in due time.