AGGRESSIVE NATURAL-KILLER-CELL LYMPHOPROLIFERATIVE DISORDER ASSOCIATED WITH EPSTEIN-BARR VIRAL-RNA

Lymphoproliferative disorder of natural killer cells is a heterogeneou s disorder, and an association with Epstein-Barr virus (EBV) is sugges ted in some cases, A Japanese male presenting with recurrent nasophary ngeal problems developed fever, generalized lymphadenopathy, and hepat osplenomegaly. Separated cells from lymph nodes were shown to have a n atural killer (NK) cell, CD2(+), CD3(-), CD16(+), CD56(+), HLA-DR(+) p henotype, A progressive abnormality of hepatic function was associated with hepatorenal failure and death, A serologic study suggested react ivated EBV infection, In situ hybridization (ISH) studies showed Epste in-Barr virus-encoded RNA (EBER)-1 in lymph nodes, with lymphocytes in filtrating the liver and tissue from ethmoid sinus surgery 3 years pri or to development of obvious lymphoproliferative disease, Polymerase c hain reaction performed on lymph node DNA, using oligonucleotide prime rs specific for the EBV lymphocyte-determined membrane antigen (LYDMA) gene, revealed a single band, suggesting monoclonal proliferation of the tumor. NK activities of the lymphocytes from the lymph node and pe ripheral blood were markedly decreased. These findings suggest a close relationship between EBV infection and development of NK cell lymphop roliferative disorder. (C) 1997 Wiley-Liss, Inc.