Clinical lymphoma, myeloma & leukemia · 2025
Dismal Outcome of EBV-Positive Nodal T/NK-Cell Lymphoma: A Multicenter Retrospective Study.
СтатьяМногоцентровое исследованиеНазвание и аннотация: Перевод в очереди
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Ichikawa Satoshi, Fukuhara Noriko, Katsuoka Yuna, Ishizawa Yuki, Kamata Kosuke, Fukatsu Masahiko, Nagao Takayo, Murai Kazunori, Yokoyama Hisayuki, Tamai Yoshiko, Ikezoe Takayuki, Ichinohasama Ryo, Ito Shigeki, Takahashi Naoto, Harigae Hideo
Аннотация
BACKGROUND: Epstein-Barr virus (EBV)-positive nodal T- and natural killer (NK)-cell lymphoma (EB-nTNKL) is a newly defined, rare, and aggressive EBV-driven lymphoid malignancy that typically affects older individuals, predominantly male, of East Asian descent. It is characterized by a cytotoxic T-cell phenotype, with only a limited number of cases documented to date.
METHODS: We performed a multicenter retrospective analysis of 11 patients diagnosed with EB-nTNKL via lymph node biopsy in Japan in order to better characterize its clinicopathological features and outcomes.
RESULTS: The median age at diagnosis was 65 years (range 24-81), and most patients presented with advanced-stage (III/IV) disease, B symptoms, and high-risk International Prognostic Index scores. Elevated serum lactate dehydrogenase (median 614 U/L) and soluble interleukin-2 receptor (median 5247 U/mL) levels were observed in all cases. All cases expressed cytotoxic molecules (granzyme B and/or TIA-1), indicating a cytotoxic T/NK-cell phenotype, and 8 cases were CD8-positive. Responses to conventional chemotherapy were poor. The median overall survival was only 2.9 months, with 6 of 11 patients dying within 3 months of diagnosis.
CONCLUSIONS: EB-nTNKL represents a distinct clinicopathological entity with a fulminant clinical course and profound chemoresistance, resulting in an extremely poor prognosis. However, differentiation from other EBV-associated T/NK-cell neoplasms, particularly those arising in younger patients, remains a diagnostic challenge and warrants further clinicopathological investigation. These dismal outcomes underscore an urgent need to develop more effective therapeutic strategies.
METHODS: We performed a multicenter retrospective analysis of 11 patients diagnosed with EB-nTNKL via lymph node biopsy in Japan in order to better characterize its clinicopathological features and outcomes.
RESULTS: The median age at diagnosis was 65 years (range 24-81), and most patients presented with advanced-stage (III/IV) disease, B symptoms, and high-risk International Prognostic Index scores. Elevated serum lactate dehydrogenase (median 614 U/L) and soluble interleukin-2 receptor (median 5247 U/mL) levels were observed in all cases. All cases expressed cytotoxic molecules (granzyme B and/or TIA-1), indicating a cytotoxic T/NK-cell phenotype, and 8 cases were CD8-positive. Responses to conventional chemotherapy were poor. The median overall survival was only 2.9 months, with 6 of 11 patients dying within 3 months of diagnosis.
CONCLUSIONS: EB-nTNKL represents a distinct clinicopathological entity with a fulminant clinical course and profound chemoresistance, resulting in an extremely poor prognosis. However, differentiation from other EBV-associated T/NK-cell neoplasms, particularly those arising in younger patients, remains a diagnostic challenge and warrants further clinicopathological investigation. These dismal outcomes underscore an urgent need to develop more effective therapeutic strategies.