scholarly journals Hydroa Vacciniforme and Hydroa Vacciniforme-Like Lymphoproliferative Disorder: A Spectrum of Disease Phenotypes Associated with Ultraviolet Irradiation and Chronic Epstein–Barr Virus Infection

2020 ◽  
Vol 21 (23) ◽  
pp. 9314
Author(s):  
Chien-Chin Chen ◽  
Kung-Chao Chang ◽  
L Jeffrey Medeiros ◽  
Julia Yu-Yun Lee
Keyword(s):  
T Cell ◽  
Natural Killer ◽  
Ultraviolet Irradiation ◽  
Epstein Barr Virus ◽  
Nk Cell ◽  
Current Evidence ◽  
Barr Virus ◽  
Ebv Infection ◽  
Hydroa Vacciniforme ◽  
Epstein Barr

Hydroa vacciniforme (HV) is a rare form of photosensitivity disorder in children and is frequently associated with Epstein–Barr virus (EBV) infection, whereas HV-like lymphoproliferative disorders (HVLPD) describe a spectrum of EBV-associated T-cell or natural killer (NK)-cell lymphoproliferations with HV-like cutaneous manifestations, including EBV-positive HV, atypical HV, and HV-like lymphoma. Classic HV occurs in childhood with papulovesicules on sun-exposed areas, which is usually induced by sunlight and ultraviolet irradiation, and mostly resolves by early adult life. Unlike classic HV, atypical or severe HV manifests itself as recurrent papulovesicular eruptions in sun-exposed and sun-protected areas associated occasionally with facial edema, fever, lymphadenopathy, oculomucosal lesions, gastrointestinal involvement, and hepatosplenomegaly. Notably, atypical or severe HV may progress to EBV-associated systemic T-cell or natural killer (NK)-cell lymphoma after a chronic course. Although rare in the United States and Europe, atypical or severe HV and HV-like lymphoma are predominantly reported in children from Asia and Latin America with high EBV DNA levels, low numbers of NK cells, and T cell clones in the blood. In comparison with the conservative treatment used for patients with classic HV, systemic therapy such as immunomodulatory agents is recommended as the first-line therapy for patients with atypical or severe HV. This review aims to provide an integrated overview of current evidence and knowledge of HV and HVLPD to elucidate the pathophysiology, practical issues, environmental factors, and the impact of EBV infection.

scholarly journals Hydroa Vacciniforme and Hydroa Vacciniforme-like Lymphoproliferative Disorder: A Spectrum of Disease Phenotypes Associated with Ultraviolet Irradiation and Chronic Epstein-Barr Virus Infection

2020 ◽  
Author(s):  
Chien-Chin Chen ◽  
Kung-Chao Chang ◽  
L. Jeffrey Medeiros ◽  
Julia Yu-Yun Lee
Keyword(s):  
T Cell ◽  
Ultraviolet Irradiation ◽  
Epstein Barr Virus ◽  
Nk Cell ◽  
The United States ◽  
Current Evidence ◽  
Barr Virus ◽  
Ebv Infection ◽  
Hydroa Vacciniforme ◽  
Epstein Barr

Hydroa vacciniforme (HV) is a rare form of photosensitivity disorders in children and is frequently associated with Epstein-Barr virus (EBV) infection, whereas HV-like lymphoproliferative disorders (HVLPD) describe a spectrum of EBV-associated T-cell or NK-cell lymphoproliferations with HV-like cutaneous manifestations, including EBV-positive HV, atypical HV, and HV-like lymphoma. Classic HV occurs in childhood with vesiculopapules on sun-exposed areas, which is usually induced by sunlight and ultraviolet irradiation, and mostly resolves by early adult life. Unlike classic HV, atypical or severe HV manifests itself as recurrent papulovesicular eruptions in sun-exposed and sun-protected areas associated occasionally with facial edema, fever, lymphadenopathy, oculomucosal lesions, gastrointestinal involvement, and hepatosplenomegaly. Notably, atypical or severe HV may progress to EBV-associated systemic T-cell or natural killer (NK)-cell lymphoma after a chronic course. Although rare in the United States and Europe, atypical or severe HV and HV-like lymphoma are predominantly reported in children from Asia and Latin America with high EBV DNA levels, low numbers of NK cells, and T cell clones in the blood. In comparison with the conservative treatment used for patients with classic HV, systemic therapy such as immunomodulatory therapy is recommended as the first-line therapy for patients with atypical or severe HV. This review aims to provide an integrated, overview of current evidence and knowledge of HV and HVLPD to elucidate the pathophysiology, practical issues, environmental factors, and the impact of EBV infection.

Characterization of novel natural killer (NK)–cell and γδ T-cell lines established from primary lesions of nasal T/NK-cell lymphomas associated with the Epstein-Barr virus

Blood ◽  
2001 ◽  
Vol 97 (3) ◽  
pp. 708-713 ◽  
Author(s):  
Hiroshi Nagata ◽  
Akiyoshi Konno ◽  
Nobuhiro Kimura ◽  
Yu Zhang ◽  
Michiko Kimura ◽  
...  
Keyword(s):  
T Cell ◽  
Natural Killer ◽  
Cell Lines ◽  
Epstein Barr Virus ◽  
Nk Cell ◽  
Cell Phenotype ◽  
Γδ T Cell ◽  
Barr Virus ◽  
Genotypic Analysis ◽  
Epstein Barr

Abstract Studies on nasal T/natural killer (NK)–cell lymphoma have been hampered by its tendency to cause necrosis. Thus, the establishment of cell lines of this neoplasm would seem to be valuable. This study attempted to establish cell lines from primary lesions of this tumor, and successfully obtained 2 novel Epstein-Barr virus (EBV)–positive cell lines, SNK-6 and SNT-8, by means of high-dose recombinant interleukin 2. Flow cytometry showed that SNK-6 had an NK-cell phenotype, CD3−CD4−CD8−CD19−CD56+T-cell receptor (TCR) α/β− TCR γ/δ−, whereas SNT-8 was CD3+CD4−CD8−CD19−CD56+TCR α/β− TCR γ/δ+. These were consistent with immunophenotypes of their original tumors, and the cell lines had monoclonal EBV clones identical to ones in their original tumors. Thus, the cell lines developed from cells forming the primary lesions. Genotypic analysis showed that SNK-6 had unrearranged TCR and immunoglobulin heavy-chain genes, supporting the conclusion that SNK-6 was of NK-cell lineage. On the other hand, SNT-8 had rearranged TCR β-, γ-, and δ-chain genes, and together with its phenotype, SNT-8 proved to be a γδ T-cell line. This is the first report of the establishment of cell lines from primary lesions of nasal T/NK cell lymphomas, and the results demonstrated that there are at least 2 lineages, NK- and γδ T-cell, in this neoplasm. Moreover, it has been suggested that nasal T/NK cell lymphomas of these lineages may belong to the same clinicopathologic entity because both types of cases shared common clinical and histopathologic features.

Quantification of circulating Epstein-Barr virus (EBV) DNA in the diagnosis and monitoring of natural killer cell and EBV-positive lymphomas in immunocompetent patients

Blood ◽  
2004 ◽  
Vol 104 (1) ◽  
pp. 243-249 ◽  
Author(s):  
Wing-Yan Au ◽  
Annie Pang ◽  
Carolyn Choy ◽  
Chor-Sang Chim ◽  
Yok-Lam Kwong
Keyword(s):  
Multivariate Analysis ◽  
Natural Killer ◽  
Epstein Barr Virus ◽  
Nk Cell ◽  
Disease Stage ◽  
Tumor Biomarker ◽  
Barr Virus ◽  
Ebv Dna ◽  
Epstein Barr ◽  
Immunocompetent Patients

Abstract In Epstein-Barr-virus (EBV)–positive lymphomas in immunocompetent patients, release of EBV DNA from tumor cells into the plasma might be useful for disease monitoring and prognostication. To test this hypothesis, we quantified serially plasma EBV DNA by quantitative polymerase chain reaction in 39 cases of EBV-positive (natural killer [NK] cell, n = 23; T cell, n = 8; B cell, n = 4; Hodgkin, n = 4) lymphomas. As control, EBV DNA was undetectable in 34 cases of EBV-negative lymphomas at diagnosis and during chemotherapy. In all cases of EBV-positive lymphomas, EBV DNA was detectable (105-1010 copies/mL) at diagnosis. It paralleled the clinical course, with EBV DNA becoming undetectable at remission and remaining elevated in refractory disease. On multivariate analysis, high-presentation EBV DNA (> 7.3 × 107 copies/mL) was significantly associated with an inferior overall survival (OS). Subgroup analysis of NK cell lymphomas, the largest cohort in this study, showed that presentation EBV DNA was correlated with disease stage and lactate dehydrogenase. On multivariate analysis, high-presentation EBV DNA (> 6.1 × 107 copies/mL) was significantly associated with an inferior disease-free survival. During treatment, patients with EBV DNA that showed further increases or failed to become undetectable had significantly inferior OS. In EBV-positive lymphomas, plasma EBV DNA is valuable as a tumor biomarker and for prognostication.

Unusual presentation of an Epstein barr virus-negative extranodal natural killer/T cell lymphoma: A diagnostic dilemma

2021 ◽  
Vol 0 ◽  
pp. 1-4
Author(s):  
Sindhu Kilaru ◽  
Soumya Surath Panda ◽  
Sourav Mishra ◽  
Debahuti Mohapatra ◽  
Spoorthy Kolluri ◽  
...  
Keyword(s):  
T Cell ◽  
Natural Killer ◽  
Epstein Barr Virus ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Natural Killer T Cell ◽  
Barr Virus ◽  
Epstein Barr ◽  
Killer T Cell ◽  
Natural Killer T

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.

scholarly journals Genomic and transcriptomic landscapes of Epstein-Barr virus in extranodal natural killer T-cell lymphoma

Leukemia ◽  
2018 ◽  
Vol 33 (6) ◽  
pp. 1451-1462 ◽  
Author(s):  
Rou-Jun Peng ◽  
Bo-Wei Han ◽  
Qing-Qing Cai ◽  
Xiao-Yu Zuo ◽  
Tao Xia ◽  
...  
Keyword(s):  
T Cell ◽  
Natural Killer ◽  
Epstein Barr Virus ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Natural Killer T Cell ◽  
Barr Virus ◽  
Epstein Barr ◽  
Killer T Cell ◽  
Natural Killer T

Significance of circulating Epstein-Barr virus DNA monitoring after remission in patients with extranodal natural killer T cell lymphoma

2018 ◽  
Vol 97 (8) ◽  
pp. 1427-1436 ◽  
Author(s):  
Jinhyun Cho ◽  
Seok Jin Kim ◽  
Silvia Park ◽  
Kwai Han Yoo ◽  
Chang-Seok Ki ◽  
...  
Keyword(s):  
T Cell ◽  
Natural Killer ◽  
Epstein Barr Virus ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Natural Killer T Cell ◽  
Barr Virus ◽  
Epstein Barr ◽  
Killer T Cell ◽  
Natural Killer T

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.

Nasal Natural Killer Cell/T-Cell Lymphoma Showing Cellular Morphology Mimicking Normal Lymphocytes

2002 ◽  
Vol 126 (5) ◽  
pp. 602-605
Author(s):  
Katsuya Chinen ◽  
Yasuhiko Kaneko ◽  
Toshiyuki Izumo ◽  
Yasuo Ohkura ◽  
Osamu Matsubara ◽  
...  
Keyword(s):  
In Situ Hybridization ◽  
T Cell ◽  
Natural Killer ◽  
Epstein Barr Virus ◽  
Cell Lymphoma ◽  
Normal Lymphocyte ◽  
Lymphoma Cells ◽  
Barr Virus ◽  
Epstein Barr

Abstract We report the autopsy case of a 34-year-old Japanese man with a nasal natural killer (NK)-cell/T-cell lymphoma. The patient developed the disease at 32 years of age, and a biopsy of the nasopharynx revealed pleomorphic lymphoma cell proliferation. Radiotherapy was performed, but the patient eventually died of respiratory failure. After radiotherapy, no histologic evidence of malignancy was obtained with biopsy materials featuring lymphocytic infiltration. Autopsy studies, including in situ hybridization for Epstein-Barr virus–encoded RNA, revealed generalized infiltration of normal lymphocyte-like, UCHL-1–positive, and Epstein-Barr virus–encoded RNA–positive lymphoma cells. Monoclonal proliferation of the Epstein-Barr virus–carrying cells was verified by means of Southern blot analysis. Retrospectively, we concluded that the normal lymphocyte-like presentation of the lymphoma cells, probably influenced by radiotherapy, prevented pathologists from recognizing the lymphoma. The utility of in situ hybridization for Epstein-Barr virus–encoded RNA in identification of tumor cells is emphasized with respect to the present case.

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