Autoimmune hepatitis is a generally progressive, chronic hepatitis of unknown cause but with an autoimmune background. In fact, one clue to diagnosis autoimmune hepatitis is the presence of other diseases with autoimmune features like thyroiditis, ulcerative colitis or type 1 diabetes. Although non-specific hematologic abnormalities have been described during the course of autoimmune hepatitis we have not found agranulocytosis in association in a review of the literature. We describe a case of agranulocytosis which appeared simultaneously associated with a flare of autoimmune hepatitis. A possible autoimmune origin of the neutropenia could be extrapolated to explain the association on the basis of the nature of the underlying liver disease, temporal association, sex, positive anti-neutrophil cytoplasm antibodies, and the successful and dose-dependent effect of corticoids on both processes.
Conclusion: We report on for the first time a case of concurrent severe autoimmune hepatitis and agranulocytosis. An autoimmune common mechanism could explain both processes.