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extranodal natural killer cell lymphoma

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https://www.readbyqxmd.com/read/29291352/recurrent-ecsit-mutation-encoding-v140a-triggers-hyperinflammation-and-promotes-hemophagocytic-syndrome-in-extranodal-nk-t-cell-lymphoma
#1
Haijun Wen, Huajuan Ma, Qichun Cai, Suxia Lin, Xinxing Lei, Bin He, Sijin Wu, Zifeng Wang, Yan Gao, Wensheng Liu, Weiping Liu, Qian Tao, Zijie Long, Min Yan, Dali Li, Keith W Kelley, Yongliang Yang, Huiqiang Huang, Quentin Liu
Hemophagocytic syndrome (HPS) is a fatal hyperinflammatory disease with a poorly understood mechanism that occurs most frequently in extranodal natural killer/T cell lymphoma (ENKTL). Through exome sequencing of ENKTL tumor-normal samples, we have identified a hotspot mutation (c.419T>C) in the evolutionarily conserved signaling intermediate in Toll pathway (ECSIT) gene, encoding a V140A variant of ECSIT. ECSIT-V140A activated NF-κB more potently than the wild-type protein owing to its increased affinity for the S100A8 and S100A9 heterodimer, which promotes NADPH oxidase activity...
January 1, 2018: Nature Medicine
https://www.readbyqxmd.com/read/29279697/misdiagnosed-refractory-extranodal-natural-killer-t-cell-lymphoma-nasal-type-successfully-treated-a-case-report
#2
José Domingo Saavedra Ramírez
Extranodal natural killer/T-cell lymphoma (ENKL), nasal type, is a rare, aggressive non-Hodgkin lymphoma for which no clear standard of care has been established, particularly in the relapsed/refractory disease setting. Because of its rarity, randomized trials are not conducted specifically on ENKL, nasal type; however, case reports and small case series can provide important insights into potential new treatments. We present a case report of a patient with ENKL, nasal type (previously misdiagnosed as relapsing chronic sinusitis), whose disease progressed during multi-agent chemotherapy but responded to second-line treatment with single-agent pralatrexate...
September 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/29277360/management-of-advanced-and-relapsed-refractory-extranodal-natural-killer-t-cell-lymphoma-an-analysis-of-stem-cell-transplantation-and-chemotherapy-outcomes
#3
Jonathan E Brammer, Dai Chihara, L Michelle Poon, Paolo Caimi, Marcos de Lima, Celina Ledesma, Gabriela Rondon, Stefan O Ciurea, Yago Nieto, Michelle Fanale, Bouthaina Dabaja, Richard T Maziarz, Richard E Champlin, Chitra Hosing, Yasuhiro Oki
BACKGROUND: Extra-Nodal natural killer/T-cell lymphoma (ENKL) is a rare lymphoma representing approximately 5-10% of T-cell non-Hodgkin lymphomas diagnosed in the United States each year. Patients with advanced stage III/IV ENKL and relapsed refractory ENKL have a poor prognosis even despite aggressive therapy and stem cell transplantation (SCT). We conducted a review of the management of 37 patients with advanced-stage and relapsed/refractory ENKL in a predominantly non-Asian cohort evaluating both chemotherapy and SCT outcomes...
January 2018: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/29195613/interim-fdg-pet-imaging-in-lymphoma
#4
REVIEW
Andrea Gallamini, Colette Zwarthoed
In the present article, the authors reviewed the rationale of FDG-PET/CT performed at an interim time point during therapy (iPET), focusing on the transition from standard, anatomical assessment of tumor shrinkage with CT to document a chemotherapy response, to the use of functional imaging with PET to assess chemosensitivity of the individual tumor. The prognostic or predictive role of iPET in different lymphoma subsets has been reviewed, with particular emphasis on early and advanced-stage Hodgkin lymphoma, diffuse large B-cell lymphoma, peripheral T-cell lymphoma, extranodal natural killer/T-cell lymphoma, and primary mediastinal B-cell lymphoma...
January 2018: Seminars in Nuclear Medicine
https://www.readbyqxmd.com/read/29194798/efficacy-and-tolerance-of-geloxd-p-gemoxd-in-newly-diagnosed-nasal-type-extranodal-nk-t-cell-lymphoma-a-multicenter-retrospective-study
#5
Ji-Wei Li, Ya-Jun Li, Mei-Zuo Zhong, Xian-Ling Liu, Jin Li, Kun-Lun Li, Xi-Yu Liu, Fang Zhou, Zhou OuYang, Zhong-Yi Sun, Li-Jun Huang, Jun-Qiao He, Hui Zhou, Ping-Yong Yi
OBJECTIVES: Nasal-type extranodal natural killer NK/T-cell lymphoma (ENKTCL) is a distinct type of non-Hodgkin lymphoma with poor prognosis. This research aimed to evaluate the efficacy and safety of the GELOXD or P-GEMOXD regimens in patients with ENKTCL. METHODS: Newly diagnosed ENKTCL patients treated with either the GELOXD or the P-GEMOXD regimen were identified from three cancer centers between January 2010 and December 2016. Kaplan-Meier and Cox regression analyses were used to calculate overall survival (OS), progression-free survival (PFS), and to investigate prognostic factors...
November 30, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/29190934/red-blood-cell-distribution-width-as-a-predictor-of-survival-in-nasal-type-extranodal-natural-killer-t-cell-lymphoma
#6
Huaichao Luo, Xiaoying Quan, Xiao-Yu Song, Li Zhang, Yilin Yin, Qiao He, Shaolei Cai, Shi Li, Jian Zeng, Qing Zhang, Yu Gao, Sisi Yu
We retrospectively enrolled 191 nasal-type, extranodal natural killer/T-cell lymphoma (ENKTL) patients newly diagnosed from 2008 to 2016 at the Sichuan Cancer Hospital, in order to evaluate the relationship between disease outcomes, demographic and clinical factors, and red blood cell distribution width (RDW). C-index, fisher's exact test, univariate analysis, and cox regression analysis were applied. The median age of patients was 44 years and 134 (70%) were men. The cutoff of RDW was 46.2 fL determined by Cutoff Finder...
November 3, 2017: Oncotarget
https://www.readbyqxmd.com/read/29189695/lymphoma-involving-skin-and-subcutaneous-fat-of-the-breast-two-cases
#7
Miri Kwon, Eun Young Ko, Boo-Kyung Han, Young Hye Ko, Eun Yoon Cho, Soo Youn Cho, Shin Young Kim
Breast lymphoma is uncommon, and it usually involves breast parenchyma and ipsilateral axillary lymph nodes. We report rare cases of cutaneous T-cell lymphoma involving the skin and subcutaneous fat layers mimicking panniculitis of the breasts in a 33-year-old male patient who presented with a growing palpable mass and pain in both breasts and in 17-year-old female patient who presented with an indolent palpable breast mass for 1 year. Extranodal natural killer/T-cell nasal-type and subcutaneous panniculitis-like T-cell lymphomas were diagnosed after skin punch biopsies...
December 2017: Ultrasound Quarterly
https://www.readbyqxmd.com/read/29187687/-symmetrical-lesions-of-the-cerebral-peduncles-and-internal-capsules-on-mri-in-a-patient-with-extranodal-nk-t-cell-lymphoma
#8
Yuko Takeuchi, Tomotsugu Kaga, Miho Ieda, Yumi Kojima, Jun Ochiai, Chiyuki Mabuchi
A 72-year-old man was admitted to our hospital because of subacute progressive consciousness disturbance. FLAIR images showed diffuse high intensity areas in the bilateral thalamus, basal ganglia, and white matter. Chest CT revealed a mass in the right lung. The patient was diagnosed with extranodal natural killer/T cell lymphoma (ENKL) according to the results of a biopsy of the right pulmonary mass. The FLAIR images showed diffuse white matter lesions, without a mass such as lymphomatosis cerebri. On diffusion-weighted images, symmetrical high-intensity lesions of the cerebral peduncle and the internal capsule suggested high-density lymphoma cells around neurons...
November 28, 2017: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/29175858/gelsolin-regulates-proliferation-apoptosis-and-invasion-in-nk-t-cell-lymphoma-cells
#9
Yanwei Guo, Hongqiao Zhang, Xin Xing, Lijuan Wang, Jian Zhang, Lin Yan, Xiaoke Zheng, Mingzhi Zhang
The expression of gelsolin (GSN) is abnormal in many cancers, including extranodal nasal-type natural killer/T-cell lymphoma (NKTCL). However, the biological function of GSN and its mechanism in NKTCL remain unclear. We found GSN overexpression significantly suppressed cell proliferation, colony formationand invasion and promoted apoptosis of YTS cells. Moreover, the upregulation of GSN significantly decreased the protein levels of PI3K and p-AKT. Interestingly, blocking the PI3K/AKT signaling pathway significantly inhibited cell proliferation and invasion and promoted apoptosis of YTS cells...
November 24, 2017: Biology Open
https://www.readbyqxmd.com/read/29063643/primary-central-nervous-system-extranodal-nasal-type-natural-killer-t-cell-lymphoma-with-cd20-expression
#10
Dujuan Li, Fangfang Fu, Lifei Lian
We report a unique case of primary CNS extranodal natural killer/T-cell lymphoma (PCNS ENKTCL) with CD20 expression and the monoclonal rearrangement of Ig heavey chain (IgH) gene. Resection specimens were evaluated using HE-stained sections, immunohistochemistry, in situ hybridization and PCR. Histopathologic examination, immunohistochemistry and molecular studies showed the intermediate-sized lymphoma cells expressing CD2, CD3ε, granzyme B, TIA-1, CD20 and Epstein-Barr virus-encoded RNA, with germline T-cell receptor gene and the monoclonal rearrangement of IgH gene...
October 24, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29056540/a-phase-ii-study-of-methotrexate-etoposide-dexamethasone-and-pegaspargase-sandwiched-with-radiotherapy-in-the-treatment-of-newly-diagnosed-stage-ie-to-iie-extranodal-natural-killer-t-cell-lymphoma-nasal-type
#11
Peng-Peng Xu, Jie Xiong, Shu Cheng, Xia Zhao, Chao-Fu Wang, Gang Cai, Hui-Juan Zhong, Heng-Ye Huang, Jia-Yi Chen, Wei-Li Zhao
BACKGROUND: A phase II study of methotrexate, etoposide, dexamethasone, and pegaspargase (MESA) sandwiched with radiotherapy for newly diagnosed, stage IE-IIE extranodal natural-killer/T-cell lymphoma, nasal-type (ENKTL) was conducted to explore its clinical efficacy and safety, as well as novel serum biomarkers upon anti-metabolic treatment. METHODS: Four cycles of MESA sandwiched with radiotherapy were administered. The primary end point was the overall response rate (ORR)...
November 2017: EBioMedicine
https://www.readbyqxmd.com/read/29025287/-incidence-and-treatment-of-extranodal-natural-killer-t-cell-lymphoma-nasal-type-hungarian-experiences
#12
Annamária Bakos, Árpád Szomor, Tamás Schneider, Zsófia Miltényi, Imelda Marton, Zita Borbényi, Judit Pammer, László Krenács, Enikő Bagdi, Klára Piukovics
INTRODUCTION: Extranodal natural killer/T (NK/T) cell lymphoma, nasal type (ENKTL) represents a rare subtype of T-cell lymphomas with aggressive clinical behavior according to WHO 2016 classification. AIM: ENKTL has distinctive geographic distribution with higher incidence in Asia and Latin America (10% of all non-Hodgkin lymphoma cases), than in Europe and North America (<1%). ENKTL tipically origins from nasopharynx and upper aerodigestive tract. Anthracycline-based chemotherapy regimens are largely ineffective in the treatment of ENKTL...
October 2017: Orvosi Hetilap
https://www.readbyqxmd.com/read/28980517/comparison-of-chemoradiotherapy-with-radiotherapy-alone-for-early-stage-extranodal-natural-killer-t-cell-lymphoma-nasal-type-in-elderly-patients
#13
Chang Su, Kevin A Nguyen, Harrison X Bai, Dhikshitha Balaji, Ya Cao, Giorgos Karakousis, Paul J Zhang, Guiying Zhang, Rong Xiao
It remains controversial whether concurrent or subsequent chemoradiotherapy (CRT) provides additional survival benefits when compared to radiotherapy (RT) alone in localized extranodal natural killer/T-cell lymphoma (ENKTL), nasal type We identified 248 patients from the US National Cancer Data Base who were diagnosed with localized ENKTL from 2004-2014; 68.9% received CRT and 31.1% received RT alone. Over time, the use of CRT increased, while the use of RT alone decreased (p < .001). On multivariate analysis, CRT was associated with longer OS than RT alone (HR: 0...
October 5, 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28947989/intensity-modulated-radiotherapy-has-superior-outcomes-to-three-dimensional-conformal-radiotherapy-in-patients-with-stage-ie-iie-extranodal-nasal-type-natural-killer-t-cell-lymphoma
#14
Yi-Yang Li, Hai-Qun Lin, Lu-Lu Zhang, Ling-Ling Feng, Shao-Qing Niu, Han-Yu Wang, Yu-Jing Zhang, Xi-Cheng Wang
We compared the treatment outcomes, toxicities and prognoses of patients with stage IE-IIE extranodal natural killer/T-cell lymphoma (ENKTL) treated with intensity-modulated radiotherapy (IMRT) or three-dimensional conformal radiotherapy (3DCRT). Newly diagnosed early-stage ENKTL patients (N = 173) were enrolled and received extended involved-field radiotherapy following induction chemotherapy. Patients were treated with 3DCRT (n = 98) or IMRT (n = 75). One-to-one matching of the IMRT and 3DCRT groups was performed through propensity score matching, which yielded 23 pairs of patients...
September 1, 2017: Oncotarget
https://www.readbyqxmd.com/read/28937369/study-of-association-of-epstein-barr-virus-in-lymphomas-by-epstein-barr-virus-encoded-rna-in-situ-hybridization-an-indian-perspective-from-a-tertiary-care-cancer-institute
#15
Roshani Gala, Jatin S Gandhi, Gurudutt Gupta, Shrruti K Grover, Anila Sharma, Sunil Pasricha, Anurag Mehta
BACKGROUND: The Epstein-Barr virus (EBV), also called human herpesvirus 4, is a virus of the herpes family. The EBV-associated lymphomas include Burkitt lymphoma, classic Hodgkin lymphoma (HL), lymphomas arising in immunocompromised individuals, peripheral T-cell lymphomas, angioimmunoblastic T-cell lymphoma, extranodal nasal-type natural killer/T-cell lymphoma, and other rare histotypes. OBJECTIVE: The present study evaluated the role of EBV as an etiologic agent in various lymphomas and determined an Indian perspective in a tertiary care cancer center compared to that of Western literature...
July 2017: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/28928855/association-between-extranodal-natural-killer-t-cell-lymphoma-and-hepatitis-b-viral-infection-a-case-control-study
#16
Kefeng Wang, Hang Yang, Wenjun He, Yi Xia, Zhongjun Xia, Su Li, Huiqiang Huang, Zhiming Li, Panpan Liu, Wenqi Jiang
Extranodal natural killer/T-cell lymphoma, nasal type (ENKTL) is a rare subtype of lymphoma that is often associated with poor clinical prognosis. Several studies have shown that hepatitis B virus (HBV) infection may be associated with increased risk of B-cell non-Hodgkin lymphoma; however, because of the rarity of ENKTL, little is known about its association with HBV. Our study aimed to assess whether HBV infection was associated with increased odds of ENKTL. We conducted a hospital-based case-control study including 417 ENKTL cases and 488 age- and sex-matched subjects with nonmalignant diseases unrelated to HBV infection...
2017: Journal of Cancer
https://www.readbyqxmd.com/read/28911074/beyond-first-line-non-anthracycline-based-chemotherapy-for-extranodal-nk-t-cell-lymphoma-clinical-outcome-and-current-perspectives-on-salvage-therapy-for-patients-after-first-relapse-and-progression-of-disease
#17
S H Lim, J Y Hong, S T Lim, H Hong, J Arnoud, W Zhao, D H Yoon, T Tang, J Cho, S Park, Y H Ko, S J Kim, C Suh, T Lin, W S Kim
Background: Current standard treatment, including non-anthracycline-based chemotherapy and optimal combining of radiotherapy, has dramatically improved outcomes of patients with extranodal natural killer/T-cell lymphoma (ENKTL) during the last decade. This study was conducted to investigate the clinical outcome of ENKTL patients with relapsed or progressive disease after initial current standard therapy. Patients and methods: We retrospectively reviewed patients diagnosed with ENKTL at six centers in four countries (China, France, Singapore, and South Korea) from 1997 to 2015 and analyzed 179 patients who had relapsed or progressed after initial current standard therapy...
September 1, 2017: Annals of Oncology: Official Journal of the European Society for Medical Oncology
https://www.readbyqxmd.com/read/28887511/hemoglobin-level-a-prognostic-factor-for-nasal-extranodal-natural-killer-t-cell-lymphoma-patients-from-stage-i-to-iv-a-validated-prognostic-nomogram
#18
Jianzhong Cao, Shengmin Lan, Liuhai Shen, Hongwei Si, Huan Xiao, Qiang Yuan, Xue Li, Hongwei Li, Ruyuan Guo
Although nasal extranodal natural killer/T-cell lymphoma (nasal ENKL) shares some prognostic factors with other lymphomas, seldom studies had explored the prognostic value of hemoglobin. The ENKL cases in stage I-IV during 2000 to 2015 were collected from two medical centers (group A, n = 192), and were randomly divided into the group B (n = 155) and C (n = 37). Although the significant factors identified by the univariate analysis differed between the group A and B, the multivariate Cox regression indicated the same factors...
September 8, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28877678/extranodal-natural-killer-t-cell-lymphoma-in-malawi-a-report-of-three-cases
#19
Tamiwe Tomoka, Eric Powers, Toon van der Gronde, Amy Amuquandoh, Bal Mukunda Dhungel, Coxcilly Kampani, Steve Kamiza, Nathan D Montgomery, Yuri Fedoriw, Satish Gopal
BACKGROUND: Extranodal NK/T-cell lymphoma (ENKTCL) reports from sub-Saharan Africa (SSA) are remarkably rare, despite early childhood acquisition and high prevalence of the causative infectious agent, Epstein-Barr virus (EBV), and frequent occurrence of other lymphoproliferative disorders causally associated with EBV. CASE PRESENTATIONS: At a national teaching hospital in Malawi, three patients of African descent were seen with ENKTCL between 2013 and 2014. Patients were aged between 29 and 60 years, two with craniofacial involvement and one with a primary abdominal tumor, and all were HIV-negative...
September 6, 2017: BMC Cancer
https://www.readbyqxmd.com/read/28875622/primary-pulmonary-extranodal-natural-killer-t-cell-lymphoma-nasal-type-presenting-as-diffuse-ground-glass-opacities-a-case-report
#20
MyungJin Song, Ji Ye Kim, Ji Soo Choi, Bora Yoon, MooHyun Kim, Soo Jeong Kim, Song Yee Kim
Extranodal natural killer (NK)/T-cell lymphoma, nasal type (ENKTCL) is a rare type of lymphoma that accounts for only 5%-18% of all cases of non-Hodgkin lymphoma (NHL). In published series, 60%-90% of NK/T-cell lymphomas are localized to the nasal and upper airway. We describe a 55-year man who presented with cough, sputum, dyspnea on exertion, and a chest computed tomography scan shows diffuse ground glass opacities (GGOs), suggestive of an interstitial lung disease. He was treated with a corticosteroid and his symptoms improved...
October 2017: Journal of Korean Medical Science
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