Lymphomatoid granulomatosis is the only form of pulmonary angiitis histolog
ically characterized by a necrotizing angiocentric and angiodestructive lym
phoid infiltrate, with an admixed T-cell reaction. We evaluated three patie
nts with a single lung nodule not diagnosed by routine radiological and end
oscopic assays. Our investigations showed a prevalence of T-cells in areas
of diffuse infiltration, which were actively replacing reaction follicular
areas of B-cells, similarly to T-cell lymphomas. Further pathologic assays
suggested the histologic diagnosis of grade I lymphomatoid granulomatosis f
or all three evaluated specimens. After two years, patients treated with a
combination of surgical resection and chemotherapy were disease free, suppo
rting the efficacy of aggressive therapy in the management of this often mi
streated group of lymphoid proliferations.