Malakoplakia of liver: report of two cases.

Malakoplakia is an unusual chronic inflammatory condition characterized by the presence of Michaelis-Gutmann bodies. Patients with malakoplakia often have an immunodeficiency state. It is believed that malakoplakia results from a defective macrophage response to phagocytosed bacteria. Malakoplakia most commonly affects the genitourinary tract. Cases confined to the liver are rare, with only five cases described in the literature. We report two cases of malakoplakia of liver; both were incidental autopsy findings. The first case involves a 53-year-old man with systemic lupus erythematosus and chronic refractory pancytopenia who presented with febrile neutropenia. His blood culture was positive for Stenotrophomonas maltophilia and Enterococcus faecium, and he subsequently developed invasive pulmonary aspergillosis. The second case involves a 60-year-old man who presented with a mass in periorbital tissue which, on biopsy, showed inflammation and Treponema-like spirochetes. He died unexpectedly at home. Autopsy revealed adrenal gland chronic inflammation and abscess. Both cases had grossly normal livers with microscopic findings of calcified targetoid structures consistent with Michaelis-Gutmann bodies. In these cases, malakoplakia was an incidental finding confined to liver. Although asymptomatic in these cases, diagnosis in the liver may be useful to initiate a search for hepatic or non-hepatic infections.