[Malignant lymphoma of the lung aassociated with Evans' syndrome: report of a case]

A 77 year-old woman who had been treated for Evans' syndrome was admitted to our hospital because of the development of bilaterally-enlarging abnormal lung shadows. She had been diagnosed as having Evans' syndrome since she was 64 years old, and had been treated with corticosteroids. She had also been found positive for hepatitis C virus, and was suffering from chronic hepatitis. The pathological examination of a transbronchial biopsy specimen revealed B-cell malignant lymphoma of the diffuse large-cell type. A computed tomography (CT) scan showed lung tumors, hilar lymph node swelling, but no other definite tumors. Gallium scintigraphy showed positive accumulation only in the lungs, and so a diagnosis of primary pulmonary lymphoma was made. She received several courses of standard CHOP therapy and achieved a partial remission. She died 8 months later, however, of bleeding from esophageal varices associated with liver cirrhosis. To our knowledge, primary pulmonary lymphoma associated with Evans' syndrome is extremely rare. Although the underlying mechanism remains to be clarified, it is likely that immunological disorders complicating Evans' syndrome lead to the development of malignant lymphoma in some cases.