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Four patients with sarcoidosis who had clinical and laboratory evidence of chronic T-cell lymphocytic activation but no evidence of opportunistic infection or malignancy were studied for a decade before they had the disease or at its onset. Patients 1 and 2 had received 18 months of chlorambucil treatment for sarcoidosis without improvement. These patients had subsequent development of malignant lymphoma. Patient 3 had a renal sarcoidosis treated with methysergide, and this patient developed Hodgkin's disease 16 months later. Patient 4 had pulmonary disease that progressed to bronchopulmonary dysplasia and died of pulmonary infection. Examination of the pathologic specimens in three of the four patients revealed a lymphocytic infiltrate consisting of predominantly CD2-, CD3+, CD4+, CD8- and T-cell receptor (TCR) V beta 11+ T cells. In addition, two of the three patients had skin lesions that had the histologic features of skin graft-versus-host disease (GVHD). 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