Lymphomatoid Granulomatosis

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Abstract
Fifteen patients with lymphomatoid granulomatosis were studied prospectively over a 10-yr period. Thirteen of the patients received the therapeutic protocol of cyclophosphamide (2 mg per kilogram of body weight per day) and prednisone (1 mg per kilogram on alternate days). Previous reports had indicated that mortality from lymphomatoid granulomatosis was as high as 90%. Of the 13 patients who received the cyclophosphamide and prednisone protocol, 7 had complete remissions lasting for 5.2 .+-. 0.6 yr (mean .+-. SEM). Six of the 7 with disease in remission have received no therapy for 28.3 .+-. 5.7 mo. Malignant lymphoma developed in 7 of the 8 who died, and only 2 of the 8 had therapy for an adequate period. Since virtually all patients who did not have complete remission went on to have malignant lymphoma, early recognition and prompt treatment during the lymphomatoid-granulomatosis phase of disease may not only lead to complete remissions but may also prevent the development of a lymphoid neoplasm.