Seronegative autoimmune hepatitis and severe aplastic anemia: Incidental concurrence or immunological consequence?

Background: Hepatitis-associated aplastic anemia (HAA) is an uncommon variant of bone marrow failure preceded by acute hepatitis. This disease usually affects previously healthy children and young adolescents. Although the etiology of HAA has been reported in association with various viral infections, the causative factor in the majority of cases remains unknown. The effectiveness of immunosuppressive regimens suggests autoimmunity as a key mechanism of HAA. However, there is a significant number of patients requiring bone marrow transplantation due to severe, intractable, and drug-resistant bone marrow hypocellularity. Case Report: The case of a 29-year-old woman with fatal HAA is reported in whom liver disease was diagnosed as seronegative autoimmune hepatitis (SAIH). A molecular hypothesis is proposed to explain this hepato-medullar syndrome. Conclusions: The major pathogenic mechanisms leading to liver injury and bone marrow destruction in HAA patients seem to have an immune nature. Patients with SAIH should be carefully monitored, and in the presence of cytopenia, directed to a hematology department.