A case of acute autoimmune hepatitis complicated by autoimmune neutropenia and autoimmune hemolytic anemia

An 84-year-old woman was admitted to her referring hospital after blood tests revealed severe liver dysfunction. During follow-up, her neutrophil count, which had been normal at admission, significantly decreased, prompting transfer to our hospital. Laboratory evaluation revealed profound neutropenia (22/μL) and persistent severe liver dysfunction. An antinuclear antibody titer of 1:320 and elevated immunoglobulin G levels raised concern for autoimmune hepatitis (AIH). Her anemia subsequently worsened, with hemoglobin decreasing to 6.9 g/dL. Haptoglobin was undetectable, and the direct Coombs test was positive. She was diagnosed with autoimmune neutropenia and warm autoimmune hemolytic anemia complicated by acute AIH. Prednisolone (PSL) 40 mg (0.8 mg/kg) was initiated for AIH, resulting in rapid improvement in liver function and neutrophil count, along with gradual improvement in anemia. After tapering PSL to 5 mg, no recurrence of hepatitis or cytopenia was observed, and the direct Coombs test became negative. This represents an extremely rare clinical presentation, which we report along with a brief review of literature.