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

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.

Author(s):  
Sindhu Kilaru ◽  
Soumya Surath Panda ◽  
Sourav Mishra ◽  
Debahuti Mohapatra ◽  
Spoorthy Kolluri ◽  
...  

Extra-nodal Natural killer/T cell lymphoma (ENKTL) is a well-defined and highly aggressive form of NonHodgkin’s lymphoma with a scarcity of cases reported in literature. The most common primary site of involvement is the nasal cavity followed by skin and the gastrointestinal tract (GIT). Cutaneous involvement is a rarity. More than 95% of cases are usually in association with Epstein Barr Virus (EBV) infection. EBV negative ENKTL can be similar in clinical, pathological, and prognostic characteristics with EBV positive ENKTL. This malignancy is usually characterized by its poor prognosis irrespective of clinical stage and therapy. We describe here, a 58-year-old man presenting with multiple nodular lesions over legs and trunk, had an ileal perforation later, and was diagnosed as ENKTL on the ileal biopsy specimen. This case is being reported in view of the fulminant clinical course of the disease, simultaneous involvement of the GIT and skin without nasal or midline involvement, the usefulness of immunohistochemistry in arriving at a diagnosis, and EBV negativity which is quite rare in the Asian population.


Leukemia ◽  
2018 ◽  
Vol 33 (6) ◽  
pp. 1451-1462 ◽  
Author(s):  
Rou-Jun Peng ◽  
Bo-Wei Han ◽  
Qing-Qing Cai ◽  
Xiao-Yu Zuo ◽  
Tao Xia ◽  
...  

2018 ◽  
Vol 23 (2) ◽  
pp. 1509-1516
Author(s):  
Weili Xue ◽  
Weiming Li ◽  
Yufeng Shang ◽  
Yanjie Zhang ◽  
Xuan Lan ◽  
...  

2018 ◽  
Vol 97 (8) ◽  
pp. 1427-1436 ◽  
Author(s):  
Jinhyun Cho ◽  
Seok Jin Kim ◽  
Silvia Park ◽  
Kwai Han Yoo ◽  
Chang-Seok Ki ◽  
...  

2017 ◽  
Vol 56 (11) ◽  
pp. 1409-1414 ◽  
Author(s):  
Ayano Imai ◽  
Hiroshi Takase ◽  
Ken-Ichi Imadome ◽  
Go Matsuda ◽  
Iichiro Ohnishi ◽  
...  

2019 ◽  
Vol 15 (10) ◽  
pp. 513-520 ◽  
Author(s):  
Pamela B. Allen ◽  
Mary Jo Lechowicz

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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