scholarly journals EBV and the Pathogenesis of NK/T Cell Lymphoma

Cancers ◽  
2021 ◽  
Vol 13 (6) ◽  
pp. 1414
Author(s):  
Ivonne A. Montes-Mojarro ◽  
Falko Fend ◽  
Leticia Quintanilla-Martinez
Keyword(s):  
T Cell ◽  
Nk Cell ◽  
Cell Lymphoma ◽  
Gene Mutations ◽  
T Cell Lymphoma ◽  
Sequencing Analysis ◽  
Barr Virus ◽  
Base Pair Deletion ◽  
Nk T Cell ◽  
Epstein Barr

Epstein-Barr virus (EBV) is a ubiquitous gamma herpes virus with tropism for B cells. EBV is linked to the pathogenesis of B cell, T cell and NK cell lymphoproliferations, with extranodal NK/T cell lymphoma, nasal type (ENKTCL) being the prototype of an EBV-driven lymphoma. ENKTCL is an aggressive neoplasm, particularly widespread in East Asia and the native population of Latin America, which suggests a strong genetic predisposition. The link between ENKTCL and different populations has been partially explored. EBV genome sequencing analysis recognized two types of strains and identified variants of the latent membrane protein 1 (LMP1), which revealed different oncogenic potential. In general, most ENKTCL patients carry EBV type A with LMP1 wild type, although the LMP1 variant with a 30 base pair deletion is also common, especially in the EBV type B, where it is necessary for oncogenic transformation. Contemporary high-throughput mutational analyses have discovered recurrent gene mutations leading to activation of the JAK-STAT pathway, and mutations in other genes such as BCOR, DDX3X and TP53. The genomic landscape in ENKTCL highlights mechanisms of lymphomagenesis, such as immune response evasion, secondary to alterations in signaling pathways or epigenetics that directly or indirectly interfere with oncogenes or tumor suppressor genes. This overview discusses the most important findings of EBV pathogenesis and genetics in ENKTCL.

scholarly journals Epstein–Barr Virus-Associated T- and NK-Cell Lymphoproliferative Diseases: A Review of Clinical and Pathological Features

Cancers ◽  
2021 ◽  
Vol 13 (13) ◽  
pp. 3315
Author(s):  
Charlotte Syrykh ◽  
Sarah Péricart ◽  
Claire Lamaison ◽  
Frédéric Escudié ◽  
Pierre Brousset ◽  
...  
Keyword(s):  
T Cell ◽  
Epstein Barr Virus ◽  
Who Classification ◽  
Nk Cell ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
World Health ◽  
Barr Virus ◽  
Nk T Cell ◽  
Epstein Barr

Epstein–Barr virus (EBV) is a ubiquitous virus detected in up to 95% of the general population. Most people are asymptomatic, while some may develop a wide range of EBV-associated lymphoproliferative disorders (LPD). Among them, EBV-positive T/NK LPD are uncommon diseases defined by the proliferation of T- or NK-cells infected by EBV. The 2017 World Health Organization (WHO) classification recognizes the following entities characterized by different outcomes: chronic active EBV infection of T- or NK-cell types (cutaneous and systemic forms), systemic EBV-positive T-cell lymphoma of childhood, EBV-positive aggressive NK-cell leukemia, extra nodal NK/T-cell lymphoma nasal type, and the new provisional entity known as primary EBV-positive nodal T/NK-cell lymphoma. In addition, EBV associated-hemophagocytic lymphohistiocytosis is part of EBV-positive T/NK LPD, but has not been included in the WHO classification due to its reactive nature. Despite novel insights from high-throughput molecular studies, EBV-positive NK/T-cell LPD diagnoses remain challenging, especially because of their rarity and overlap. Until now, an accurate EBV-positive NK/T LPD diagnosis has been based on its clinical presentation and course correlated with its histological features. This review aims to summarize clinical, pathological and molecular features of EBV-positive T/NK LPD subtypes and to provide an overview of new understandings regarding these rare disorders.

scholarly journals Progress in the treatment of NK-cell lymphoma/leukemia

2021 ◽  
Author(s):  
Ayumi Fujimoto ◽  
Ritsuro Suzuki
Keyword(s):  
T Cell ◽  
Nk Cell ◽  
Cell Lymphoma ◽  
Genetic Alterations ◽  
T Cell Lymphoma ◽  
Barr Virus ◽  
T Cell Lymphomas ◽  
Previous Decade ◽  
Nk T Cell ◽  
Epstein Barr

Natural killer (NK)/T cell lymphoma includes two major subtypes of disease, specifically extranodal NK/T cell lymphoma, nasal type (ENKL) and aggressive NK cell leukemia (ANKL). Both are strongly associated with Epstein-Barr virus and are prevalent in East Asia and Latin America. Except for that of limited-stage ENKL, the prognosis of both diseases was poor in the previous decade. The advent of non-anthracycline-based chemoradiotherapy has contributed to an improvement in ENKL prognosis, but there is still room for further treatment progress. Recently, the high efficacy of PD-1 antibody was reported in relapsed or refractory ENKL patients. This was later supported by the finding that PD-L1/PD-L2 genetic alterations are frequently observed in ENKL and ANKL patients. Due to the rarity of the disease, a standard treatment for ANKL remains to be established. Currently, allogeneic stem cell transplantation is the only curative treatment, and this is even applicable to chemo-resistant ANKL patients. In this review, we focus on recent treatment approaches for NK/T cell lymphomas including novel agents.

scholarly journals Epidemiology and Pathogenesis of Nasal NK/T-Cell Lymphoma: A Mini-Review

2011 ◽  
Vol 11 ◽  
pp. 422-428 ◽  
Author(s):  
Katsuyuki Aozasa ◽  
Mona A. A. Zaki
Keyword(s):  
T Cell ◽  
Latin American ◽  
Cell Lymphoma ◽  
Gene Mutations ◽  
T Cell Lymphoma ◽  
Upper Respiratory Tract ◽  
Case Control Studies ◽  
Hla Antigen ◽  
Barr Virus ◽  
Nk T Cell

Nasal NK/T-cell lymphoma (NKTCL) frequently presents with necrotic, granulomatous lesions in the upper respiratory tract, and usually shows a highly aggressive clinical course. Thus, it was initially included in the clinical condition of lethal midline granuloma. Recently, the disease has been recognized as a neoplastic proliferation of NK/T cells. The disease is much more frequent in Asian and Latin American countries than in Western countries, and is universally associated with Epstein-Barr virus (EBV) infection. Analyses of gene mutations, especially p53 and c-kit, revealed the different frequencies by district. Abnormalities of other genes have also been reported. Case-control studies showed that the exposure to pesticides and chemical solvents could be causative of NKTCL. Further studies including HLA antigen typing of patients is necessary to further clarify the disease mechanism.

scholarly journals One method to establish Epstein-Barr virus-associated NK/T cell lymphoma mouse models

2018 ◽  
Vol 23 (2) ◽  
pp. 1509-1516
Author(s):  
Weili Xue ◽  
Weiming Li ◽  
Yufeng Shang ◽  
Yanjie Zhang ◽  
Xuan Lan ◽  
...  
Keyword(s):  
T Cell ◽  
Mouse Models ◽  
Epstein Barr Virus ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Barr Virus ◽  
Nk T Cell ◽  
Epstein Barr

Epstein-Barr virus-induced natural killer/T cell lymphoma arising in tonsil and cervical node tissue

2021 ◽  
Vol 14 (11) ◽  
pp. e235100
Author(s):  
Adora Tricia Santos ◽  
Jiankun Tong ◽  
Amir Steinberg ◽  
Larry Shemen
Keyword(s):  
T Cell ◽  
Natural Killer ◽  
Epstein Barr Virus ◽  
Cell Lymphoma ◽  
Early Recognition ◽  
T Cell Lymphoma ◽  
Unique Type ◽  
Barr Virus ◽  
Nk T Cell ◽  
Epstein Barr

Infection with Epstein-Barr virus (EBV) has been linked to approximately 10%–15% of lymphomas diagnosed in the USA, including a small percentage of Natural Killer (NK)/T cell lymphomas, which are clinically aggressive, respond poorly to chemotherapy and have a shorter survival. Here, we present a case of a patient found to have EBV-induced NK/T cell lymphoma from a chronic EBV infection. While the EBV most commonly infects B cells, it can infect NK/T cells, and it is important for the clinician to be aware of the potential transformation to lymphoma as it is clinically aggressive, warranting early recognition and treatment. NK/T cell lymphoma is a unique type of non-Hodgkin's lymphoma that is almost always associated with EBV. The disease predominantly localises in the upper aerodigestive tract, most commonly in the nose.

scholarly journals Development of Extranodal NK/T-cell Lymphoma Nasal Type in Cerebrum Following Epstein-Barr Virus-positive Uveitis

2017 ◽  
Vol 56 (11) ◽  
pp. 1409-1414 ◽  
Author(s):  
Ayano Imai ◽  
Hiroshi Takase ◽  
Ken-Ichi Imadome ◽  
Go Matsuda ◽  
Iichiro Ohnishi ◽  
...  
Keyword(s):  
T Cell ◽  
Epstein Barr Virus ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Nasal Type ◽  
Barr Virus ◽  
Nk T Cell ◽  
Epstein Barr

scholarly journals Management of NK/T-Cell Lymphoma, Nasal Type

2019 ◽  
Vol 15 (10) ◽  
pp. 513-520 ◽  
Author(s):  
Pamela B. Allen ◽  
Mary Jo Lechowicz
Keyword(s):  
T Cell ◽  
Epstein Barr Virus ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Nasal Type ◽  
Barr Virus ◽  
Limited Stage ◽  
Stage Disease ◽  
Nk T Cell ◽  
Epstein Barr

Extranodal natural killer (NK)/T-cell lymphoma, nasal type, is a rare peripheral T-cell lymphoma associated with Epstein-Barr virus. It most often presents as limited-stage disease in patients of East Asian descent with a palatal deformity caused by erosion of the tumor through the hard palate. Limited-stage disease is often curable with the use of l-asparaginase–based chemotherapy and high-dose radiation therapy. Obtaining an accurate diagnosis is essential, because treatment with standard lymphoma regimens and omission of radiation severely compromise the likelihood of long-term survival. Conversely, patients with advanced disease have a poor prognosis and are recommended for asparaginase-based chemotherapy followed by consolidation with autologous transplantation as a potentially curative approach. Progress often has been hampered by the rarity of this disease. However, discovery of common genetic alterations in pathways that promote growth and inhibit apoptosis, and actionable markers such as CD30 (among others), have begun to broaden the availability of novel drugs (eg, targeted therapies). There is also cautious optimism about immunotherapies, such as checkpoint blockade and novel cellular therapies that target Epstein-Barr virus. Advances in treatment and understanding of the genetic landscape of this disease offer hope for improved treatment outcomes.

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