- Autoimmune hepatitis (AIH) is a progressive, chronic disease of unknown cause with varying presenting symptoms, ranging from no symptoms through nonspecific symptoms to fulminant hepatic failure. Although nonspecific hematologic abnormalities in AIH may occur, a case of agranulocytosis (<100 neutrophils/microL) associated with a flare of AIH and suspected to be of autoimmune origin was recently reported. Increased levels of suppressing cytokines had been previously reported in bone marrow samples of patients with AIH type-1 (AIH-1). These changes could be related to induction of apoptosis or interference with differentiation and proliferation of the myeloid lineage, hence, playing a meaningful role in the pathogenesis of agranulocytosis in patients with AIH-1. Here, we report a patient with agranulocytosis at first presentation of AIH-1. On the basis of the patient's diagnostic evaluation, response to administered therapy, and the review of the literature, we suggest several possible mechanisms relating to bone marrow cytokine milieu changes, in addition to autoimmune pathogenesis, that could explain this phenomenon.