Pure white cell aplasia: report of first case associated with autoimmune hepatitis

Pure white cell aplasia (PWCA) is a rare, immune-mediated condition that causes a profound inhibition of myelopoiesis. It has been seen in association with other autoimmune conditions, thymomas, chronic lymphocytic leukaemia and as an adverse drug reaction. We report what we believe to be the first case of PWCA associated with autoimmune hepatitis. An 18-year-old woman presented with jaundice and was found to be suffering from acute hepatitis. Later she became neutropenic and lymphopenic. Liver biopsy was indicative of autoimmune hepatitis. This was later confirmed by autoantibody results. Bone marrow aspirate found an absence of all myeloid progenitor cells, highly suggestive of PWCA. The low white cell count only improved after immunosuppression with corticosteroids. Her condition remains stable 1 year later with azathioprine therapy.