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
M. Mizuki et al., 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, American journal of hematology, 59(4), 1998, pp. 309-315
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.