Epstein-Barr virus-positive nodal T/NK-cell lymphoma: An analysis of 15 cases with distinct clinicopathological features

Yoon Kyung Jeon, Jo Heon Kim, Ji Youn Sung, Jae Ho Han, Young Hyeh Ko

Research output: Contribution to journalArticle

14 Citations (Scopus)

Abstract

Nodal peripheral T-cell lymphoma, not otherwise specified, is a heterogeneous entity with variable biologic behavior. We analyze the clinicopathological features of 15 patients with Epstein-Barr virus-positive (EBV+) nodal T/NK-cell lymphoma, including 9 males and 6 females, with a median age of 64 years. All patients presented with multiple lymphadenopathy with common B symptoms (80%, 12/15) at an advanced Ann Arbor stage (III, IV) (87%, 13/15). The International Prognostic Index was high or high/intermediate in 87% (13/15) of patients, and the prognostic index for peripheral T-cell lymphoma was group 3 or 4 in 73% (11/15). Spleen and liver involvement was observed in 73% (11/15) and 60% (9/15) of patients, respectively. In contrast, extranodal involvement was infrequent, with no more than 1 site in 71% (10/15) of patients. Moreover, none had nasal lesions, and only 1 had mucocutaneous involvement. The cell lineage of EBV+ tumor cells was determined to be T cell in all except 1 patient, who was NK-cell lineage. Cytotoxic molecules were expressed in all cases, and 64% (9/14) of patients expressed the αβT-cell receptor. Moreover, most patients (67%, 10/15) showed CD8 positivity, with 2 of them being CD4CD8 double positive; the others were CD4 positive (n = 2) or CD4CD8 double negative (n = 3). The clinical course was very aggressive, with a median survival time of 3.5 months, and 10 patients died within 6 months of diagnosis. Taken together, our data demonstrate that EBV+ nodal T/NK-cell lymphoma is a distinct clinicopathological entity characterized by cytotoxic molecule expression, a frequent CD8-positive αβT-cell lineage, and a very aggressive clinical behavior.

Original languageEnglish
Pages (from-to)981-990
Number of pages10
JournalHuman Pathology
Volume46
Issue number7
DOIs
StatePublished - 1 Jul 2015

Fingerprint

T-Cell Lymphoma
Human Herpesvirus 4
Natural Killer Cells
Cell Lineage
Peripheral T-Cell Lymphoma
T-Lymphocytes
T-Cell Antigen Receptor
Nose
Spleen
Survival
Liver

Keywords

  • Cytotoxic molecule
  • EBV positive nodal T/NK-cell lymphoma
  • Epstein-Barr virus
  • Extranodal NK/T-cell lymphoma
  • Not otherwise specified
  • Peripheral T-cell lymphoma

Cite this

Jeon, Yoon Kyung ; Kim, Jo Heon ; Sung, Ji Youn ; Han, Jae Ho ; Ko, Young Hyeh. / Epstein-Barr virus-positive nodal T/NK-cell lymphoma : An analysis of 15 cases with distinct clinicopathological features. In: Human Pathology. 2015 ; Vol. 46, No. 7. pp. 981-990.
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abstract = "Nodal peripheral T-cell lymphoma, not otherwise specified, is a heterogeneous entity with variable biologic behavior. We analyze the clinicopathological features of 15 patients with Epstein-Barr virus-positive (EBV+) nodal T/NK-cell lymphoma, including 9 males and 6 females, with a median age of 64 years. All patients presented with multiple lymphadenopathy with common B symptoms (80{\%}, 12/15) at an advanced Ann Arbor stage (III, IV) (87{\%}, 13/15). The International Prognostic Index was high or high/intermediate in 87{\%} (13/15) of patients, and the prognostic index for peripheral T-cell lymphoma was group 3 or 4 in 73{\%} (11/15). Spleen and liver involvement was observed in 73{\%} (11/15) and 60{\%} (9/15) of patients, respectively. In contrast, extranodal involvement was infrequent, with no more than 1 site in 71{\%} (10/15) of patients. Moreover, none had nasal lesions, and only 1 had mucocutaneous involvement. The cell lineage of EBV+ tumor cells was determined to be T cell in all except 1 patient, who was NK-cell lineage. Cytotoxic molecules were expressed in all cases, and 64{\%} (9/14) of patients expressed the αβT-cell receptor. Moreover, most patients (67{\%}, 10/15) showed CD8 positivity, with 2 of them being CD4CD8 double positive; the others were CD4 positive (n = 2) or CD4CD8 double negative (n = 3). The clinical course was very aggressive, with a median survival time of 3.5 months, and 10 patients died within 6 months of diagnosis. Taken together, our data demonstrate that EBV+ nodal T/NK-cell lymphoma is a distinct clinicopathological entity characterized by cytotoxic molecule expression, a frequent CD8-positive αβT-cell lineage, and a very aggressive clinical behavior.",
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Epstein-Barr virus-positive nodal T/NK-cell lymphoma : An analysis of 15 cases with distinct clinicopathological features. / Jeon, Yoon Kyung; Kim, Jo Heon; Sung, Ji Youn; Han, Jae Ho; Ko, Young Hyeh.

In: Human Pathology, Vol. 46, No. 7, 01.07.2015, p. 981-990.

Research output: Contribution to journalArticle

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T1 - Epstein-Barr virus-positive nodal T/NK-cell lymphoma

T2 - An analysis of 15 cases with distinct clinicopathological features

AU - Jeon, Yoon Kyung

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AB - Nodal peripheral T-cell lymphoma, not otherwise specified, is a heterogeneous entity with variable biologic behavior. We analyze the clinicopathological features of 15 patients with Epstein-Barr virus-positive (EBV+) nodal T/NK-cell lymphoma, including 9 males and 6 females, with a median age of 64 years. All patients presented with multiple lymphadenopathy with common B symptoms (80%, 12/15) at an advanced Ann Arbor stage (III, IV) (87%, 13/15). The International Prognostic Index was high or high/intermediate in 87% (13/15) of patients, and the prognostic index for peripheral T-cell lymphoma was group 3 or 4 in 73% (11/15). Spleen and liver involvement was observed in 73% (11/15) and 60% (9/15) of patients, respectively. In contrast, extranodal involvement was infrequent, with no more than 1 site in 71% (10/15) of patients. Moreover, none had nasal lesions, and only 1 had mucocutaneous involvement. The cell lineage of EBV+ tumor cells was determined to be T cell in all except 1 patient, who was NK-cell lineage. Cytotoxic molecules were expressed in all cases, and 64% (9/14) of patients expressed the αβT-cell receptor. Moreover, most patients (67%, 10/15) showed CD8 positivity, with 2 of them being CD4CD8 double positive; the others were CD4 positive (n = 2) or CD4CD8 double negative (n = 3). The clinical course was very aggressive, with a median survival time of 3.5 months, and 10 patients died within 6 months of diagnosis. Taken together, our data demonstrate that EBV+ nodal T/NK-cell lymphoma is a distinct clinicopathological entity characterized by cytotoxic molecule expression, a frequent CD8-positive αβT-cell lineage, and a very aggressive clinical behavior.

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