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Human cases of infection with a granulocytotropic Ehrlichia species closely related to Ehrlichia equi are now being described with increasing frequency in the United States, especially in areas where
Lyme disease is already endemic. We describe a case of fatal pancarditis during the course of human granulocytic ehrlichiosis (HGE) in a 44-year-old outdoor worker who was previously treated for presumptive
Lyme disease. Serological and molecular diagnostic tests for Borrelia burgdorferi and Babesia microti infections were negative. Postmortem serum specimens were seroreactive for HGE, and molecular evidence of infection with the HGE agent was obtained. These findings suggest that carditis may be a manifestation of HGE, further complicating the differential diagnosis of tick-borne illness.