Lymphomatoid granulomatosis: association with retroperitoneal fibrosis and evidence of impaired cell-mediated immunity.

Lymphomatoid granulomatosis is a non-neoplastic lymphoreticular disorder that usually presents as primary lung disease. A case of lymphomatoid granulomatosis associated with retroperitoneal fibrosis is reported. The retroperitoneal fibrous tissue contained the same variegated lymphoreticular infiltrate that formed the lung tumor, suggesting that the disease may have begun in the retroperitoneum. An extensive immunologic evaluation of the patient revealed impaired cell-mediated immunity.