Pulmonary lymphomatoid granulomatosis with immunodeficiency terminating as malignant lymphoma.

Lymphomatoid granulomatosis was diagnosed in a 60-yr-old woman 2 yr after presentation with a multi-system disorder resembling sarcoidosis. Five months later autopsy revealed malignant lymphoma. Large aggregates of intracytoplasmic tubular structures resembling nucleocapsid material of the paramyxovirus group were found within cells of lymphoma deposits in the liver. Sequential immunological studies over more than 2 years demonstrated a relatively stable T-cell deficiency associated with variable B-cell dysfunction. The latter was characterized by the production of immunoglobulins of restricted electrophoretic mobility. Intermittent hypercalcaemia was associated with increases in serum IgG and appeared to be due to the presence of Ca-binding paraproteins. It is suggested that lymphomatoid granulomatosis may be a pre-malignant lymphoproliferation, with immune deficiency as a predisposing cause. The pattern of immunological abnormalities suggests that the lymphoma may have been due to B-cell malignant transformation.