NATURAL-KILLER CELL-DERIVED LARGE GRANULAR LYMPHOCYTE LYMPHOMA OF LUNG DEVELOPED IN A PATIENT WITH HYPERSENSITIVITY TO MOSQUITO BITES AND REACTIVATED EPSTEIN-BARR-VIRUS INFECTION

A 17-year-old female developed natural killer (NK) cell-derived large granular lymphocyte (LGL) lymphoma of the lung. She had a past history of hypersensitivity to mosquito bites (HMB). After an eight-year chro nic, active Epstein-Barr virus (EBV) infection, she developed multiple lung lesions and pleural effusion. In the effusion, 60% of the cells were LGL, They were CD2+, 3-, 16+, 56+, 57+, 45RO+/RA + weak, and poss essed strong NK activity. No rearrangement of T-cell-receptor genes wa s detected. From all these results, a diagnosis of NK-LGL lymphoma of the lung was made. EB virus RNA was detected in cells infiltrating the pleural effusion. The clonality of the LGLs was determined by Souther n blot hybridization with the terminal repeat sequence of EB virus as a probe, and by chromosomal abnormalities. The patient died from respi ratory failure. Necropsy of the lung revealed diffuse lymphoma compose d of polymorphic cells with typical angiocentric lesions. Reportedly, lymphomas of NK lineage show predominantly extranodal involvement, and primary lung lesions are rare. In the pleural effusion of the present case, abnormally high levels of soluble Fas ligand, interleukin-10 an d interferon gamma were detected. This hypercytokinemia, reflecting th e microenvironment of lymphoma cells, may play a role in the progressi on of the lymphoma and organ injury in the lung. (C) 1998 Wiley-Liss, Inc.