Aggressive natural killer cell lymphoproliferative disorder associated with Epstein-Barr viral RNA.
Lymphoproliferative disorder of natural killer cells is a heterogeneous disorder, and an association with Epstein-Barr virus (EBV) is suggested in some cases. A Japanese male presenting with recurrent nasopharyngeal problems developed fever, generalized lymphadenopathy, and hepatosplenomegaly. Separated cells from lymph nodes were shown to have a natural killer (NK) cell, CD2(+), CD3(-), CD16(+), CD56(+), HLA-DR(+) phenotype. A progressive abnormality of hepatic function was associated with hepatorenal failure and death. A serologic study suggested reactivated EBV infection. In situ hybridization (ISH) studies showed Epstein-Barr virus-encoded RNA (EBER)-1 in lymph nodes, with lymphocytes infiltrating the liver and tissue from ethmoid sinus surgery 3 years prior to development of obvious lymphoproliferative disease. Polymerase chain reaction performed on lymph node DNA, using oligonucleotide primers 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 peripheral blood were markedly decreased. These findings suggest a close relationship between EBV infection and development of NK cell lymphoproliferative disorder.
Hirose
Y
Y
Department of Internal Medicine, Kanazawa Medical University, Kahoku-gun, Ishikawa, Japan.
Masaki
Y
Y
Yoshioka
R
R
Takiguchi
T
T
D000328:Adult; D004854:Herpesvirus 4, Human / Q000302:isolation & purification*; D006801:Humans; D017403:In Situ Hybridization; D007694:Killer Cells, Natural / Q000473:pathology / Q000821:virology*; D008232:Lymphoproliferative Disorders / Q000821:virology*; D008297:Male; D016376:Oligonucleotides, Antisense; D016133:Polymerase Chain Reaction; D012367:RNA, Viral / Q000032:analysis*
D016376:Oligonucleotides, Antisense; D012367:RNA, Viral