This report describes a case of hairy cell leukemia (HCL) occurring with autoimmune chronic active hepatitis (CAH). A 74-year-old woman presented with typical clinical and histologic features of HCL for which splenectomy was performed. 2 years later she developed abnormal liver function tests due to autoimmune CAH. The liver function tests improved promptly after prednisolone therapy. HCL has been reported to occur with several immune-mediated diseases including polyarteritis nodosa, rheumatoid arthritis, hemolytic anemia, cutaneous vasculitis and monoclonal gammopathy with amyloidosis. This report adds a further example of HCL coexisting with an immune-mediated disease, in this case autoimmune CAH.

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