1 Most cases are of B cell origin and commonly arise from bronchial mucosa associated lymphoid tissue (MALT). The only situation where radiotherapy might be used alone is in elderly patients with poor performance and significant comorbidities that preclude chemotherapy [50]. EpsteinBarr virus (EBV) infection in lymphoma cells is universal. Huang H, Zhu J, Yao M, Kim TM, Yoon DH, Cho SG, et al. 2017;41(1):6774. The World Health Organization (WHO) and revised EuropeanAmerican classification of lymphoid neoplasms (REAL) defines extranodal T cell or NK cell neoplasms based on their anatomical sites, phenotype, and genotype.14,15 Histopathological examination usually reveals cytological dysplasia, homogeneous populations of cells, and marked architectural effacement. 2018;97(1):1936. Yok-Lam Kwong. Primary lymphoma of the lung is rare and is usually of B cell type. Asia Lymphoma Study Group. The new finding of CD56+ lymphocytes increased the likelihood of an atypical lymphoproliferative process involving NK cells, but it cannot be considered pathognomonic for a malignant process. Yamaguchi M, Kita K, Miwa H, Nishii K, Oka K, Ohno T, et al. Onco Targets Ther. Allogeneic HSCT is needed for any hope of survival. Systemic corticosteroids may be tried as a temporary measure to stabilise the patient sufficiently to undergo surgical biopsy. Allogeneic haematopoietic stem cell transplantation as a promising treatment for natural killer-cell neoplasms. Blood Adv. Kim SJ, Jung HA, Chuang SS, Hong H, Guo CC, Cao J, et al. Validation of nomogram-revised risk index and comparison with other models for extranodal nasal-type NK/T-cell lymphoma in the modern chemotherapy era: indication for prognostication and clinical decision-making. Extranodal natural killer (NK)/T-cell lymphoma is an aggressive malignancy of putative NK-cell origin, with a minority deriving from the T-cell lineage. It can start in the thymus and may grow in the area between the lungs. PubMed T cell receptor (TCR) gene rearrangement was attempted but was unsuccessful due to paucity of viable cells. 2018;11:660515. Management of T-cell and natural-killer-cell neoplasms in Asia: consensus statement from the Asian Oncology Summit 2009. Similar to pembrolizumab, the anti-PD1 antibody nivolumab was also reported to be effective at low doses in relapsed/refractory NK/T-cell lymphoma [90], with all three treated patients showing response, one of whom remaining in continuous CR after nine cycles of treatment. The lung is usually involved as a metastasised organ. Siu LL, Chan JK, Wong KF, Kwong YL. 2017;10(1):69. The most frequent subtype of pulmonary NHL is low-grade B-cell lymphoma, such as lymphoma of mucosa-associated lymphoma tissue. Genomic and transcriptomic characterization of natural killer T cell lymphoma. The use of intensity-modulated radiotherapy (IMRT) decreased the radiation exposure to normal surrounding tissue and provided good tumour target coverage [54]. Primary extranodal natural killer/T- cell lymphoma, nasal type (NK/TCL) in the lung is extremely rare and associated with Epstein-Barr virus (EBV) infection. Cho J, Kim SJ, Park WY, Kim J, Woo J, Kim G, et al. This is clearly an area where more research and prospective studies are required. Epub 2001 Dec 6. 2004;104(1):2439. The anti-CD30 antibody conjugate brentuximab vedotin (BV) had been reported to be efficacious in two patients with relapsed/refractory NK/T-cell lymphomas [93, 94]. 2002;160(1):5966. Tse, E., Zhao, WL., Xiong, J. et al. Kim SJ, Yoon SE, Kim WS. J Hematol Oncol 15, 74 (2022). Risk-adapted therapy for early-stage extranodal nasal-type NK/T-cell lymphoma: analysis from a multicenter study. Examination of the nasopharynx did not show any obvious lesion. Lymphoma cells can invade blood vessels leading to the bleeding of surrounding tissues. PTCL-NOS includes the cases that do not fit any specific subtype in the current WHO classification. 2015;21:1597604. The HEA subtype (about 27% of cases) was defined by mutations in HDAC9, EP300, and ARID1A. These locations are designated as extranasal ENKTCL. Lymphoma cells had a predominant NK-cell gene expression pattern, with JAK/STAT pathway activation, programmed cell death protein ligand 1/2 (PD-L1/2) overexpression, and genomic instability. 2015;33(21):2399404. Tse E, Kwong YL. Primary cutaneous NK/T-cell lymphoma, nasal type and CD56-positive peripheral T-cell lymphoma: a cellular lineage and clinicopathologic study of 60 patients from Asia. During treatment, plasma EBV DNA should be serially monitored. Onco Targets Ther. 2017;10(1):85. These patients should be given vigorous supportive treatment and started on asparaginase-containing regimens as soon as feasible. Tian XP, Ma SY, Young KH, Ong CK, Liu YH, Li ZH, et al. Bi YW, Wang SY, Qian J, Yuan YF, Li XQ, Zhu XZ. However, these studies were seriously flawed, because the outcomes of the SMILE cohorts were exceptionally poor, which accounted for the apparent but probably erroneous superiority of the DDGP regimen. Lymphomas arising putatively from natural killer (NK) cells were first reported more than seventy years ago [1]. Oncotarget. Allogeneic haematopoietic SCT for natural killer/T-cell lymphoma: a multicentre analysis from the Asia Lymphoma Study Group. Concurrent chemoradiotherapy has been proposed for stage I/II diseases, predicated on the notion that radiosensitivity can be enhanced with concurrent chemotherapy [56]. Kanate AS, DiGilio A, Ahn KW, Al Malki M, Jacobsen E, Steinberg A, et al. PINK-E appears particularly useful, as it incorporates a biologic parameter, EBV DNA, which has been shown to be prognostic important [36,37,38,39,40]. Tse E, Chan TS, Koh LP, Chng WJ, Kim WS, Tang T, et al. Only three patients were asymptomatic and were diagnosed after incidental discovery of an abnormal chest radiograph.6,7,13 The most common radiographic finding was bilateral diffuse nodular lesions.3,5,7,9,12 Masslike consolidation,10,11,13 cryptogenic organising pneumonia (COP)like lesions,4,8 hilar adenopathy,3 and pleural effusion11 were also reported. NK/T cell lymphoma of the lung: a case report and review of literature WHO classification of tumours of haematopoietic and lymphoid tissues. All authors read and approved the final manuscript. A CT guided transthoracic needle biopsy of the right perihilar area was therefore undertaken which revealed CD3+, CD20, and CD56+ cells indicating that the cells were of T and NK cell lineages. 1eg). J Clin Oncol. Therefore, CT-guided needle biopsy, bronchoscopic examination, or even wedge biopsy should be considered when clinical symptoms show deterioration despite adequate antibiotic therapy. Stage III/IV nasal, and non-nasal and aggressive leukaemia/lymphoma cases are treated with asparaginase-containing regimens and consolidated by allogeneic haematopoietic stem cell transplantation (HSCT) in suitable patients. Genome-wide association studies in predominantly Asian patients identified three susceptibility loci, HLA-DPB1, IL18RAP, and HLA-DRB1, with genetic differences leading to amino-acid changes potentially affecting immune responses to EBV infection, thus contributing to the pathogenesis of and increasing the susceptibility to NK/T-cell lymphoma [22, 23]. Kim SJ, Yang DH, Kim JS, Kwak JY, Eom HS, Hong DS, et al. The patient underwent a repeat bronchoscopy with TBB which again indicated lymphocytic infiltration. Article 2016;127(16):200717. She refused further evaluation and developed progressive respiratory failure and died. Next-generation sequencing further defined recurrent mutations in RNA helicases, tumour suppressors, JAK-STAT pathway genes, epigenetic modifiers, and other oncogenes [16, 20, 21]. 2003 Jul;40(3):221-32. doi: 10.1016/s0037-1963(03)00136-7. the contents by NLM or the National Institutes of Health. official website and that any information you provide is encrypted Epstein Barr virus-associated lymphoproliferative diseases: the virus as a therapeutic target. Phase II study of SMILE chemotherapy for newly diagnosed stage IV, relapsed, or refractory extranodal natural killer (NK)/T-cell lymphoma, nasal type: the NK-Cell Tumor Study Group study. Bethesda, MD 20894, Web Policies PubMed A bronchoscopy with transbronchial biopsy (TBB) was performed. Kwong YL, Pang AW, Leung AY, Chim CS, Tse E. Quantification of circulating EpsteinBarr virus DNA in NK/T-cell lymphoma treated with the SMILE protocol: diagnostic and prognostic significance. In another study, four of seven patients with relapsed/refractory disease responded to pembrolizumab treatment [89]. Genetic risk of extranodal natural killer T-cell lymphoma: a genome-wide association study in multiple populations. Apart from HIV related lymphoproliferative disorders, primary lymphoma of the lung is rare.1 While extranodal manifestations of nonHodgkin's lymphoma (NHL) are not uncommon, isolated involvement of the lung is found in only 34% of cases.1 Most cases reported are B cell lymphoma.2 The true incidence of pulmonary lymphomas other than B cell type is unknown. Primary nasal natural killer cell lymphoma: long-term treatment outcome and relationship with the International Prognostic Index. Quantification of circulating EpsteinBarr virus (EBV) DNA in the diagnosis and monitoring of natural killer cell and EBV-positive lymphomas in immunocompetent patients. No prospective randomized trials have been conducted to compare how these two modalities should be sequenced or combined. 2019;9:62. Typically, the cells bear CD56 on the surface, with or without CD3.14 CD20 is usually absent. Ng SB, Yan J, Huang G, Selvarajan V, Tay JL, Lin B, et al. A more practical prognostic model developed for patients treated with non-anthracycline-containing regimens is the prognostic index for NK/T-cell lymphomas (PINK) (negative scoring parameters: age>60years, stage III/IV disease, distant lymph node involvement, non-nasal subtype), and its variant PINK-E (additional negative scoring parameter: detectable presentation EBV DNA) [46]. Lyon: International Agency for Research on Cancer; 2017. p. 36871. The BAL fluid cultures were negative. 2018;2(18):236977. Kwong YL, Kim WS, Lim ST, Kim SJ, Tang T, Tse E, et al. Sequential chemotherapy and radiotherapy are adopted in most centres, as shown by the abundance of studies employing this strategy. These findings suggest that targeting EBV might be a treatment strategy [24]. Semin Respir Crit Care Med. Similarly, end-of-treatmentdetectable circulating EBV DNA and PET/CT scan with a Deauville score of >3 had also been shown to portend inferior long-term prognosis [40], suggesting that patients with such results would require additional treatment to improve outcome. 2021 Mar 19;21(1):94. doi: 10.1186/s12890-021-01457-y. Kim SJ, Lim JQ, Laurensia Y, Cho J, Yoon SE, Lee JY, et al. 2014;55(6):9116. Prof Chng, who is the co-lead author of the study, said, "We have now identified novel treatment targets, TOX2 and the downstream PRL3, in NKTL, where new treatment is greatly needed. Chidamide is an orally active inhibitor of histone deacetylases 1, 2, 3, and 10 (HDACi) [27]. Mod Pathol. Lyon: International Agency for Research on Cancer; 2017. p. 3534. PubMed Central As a library, NLM provides access to scientific literature. 2018;11(1):134. Cite this article. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. Inhibition of the PD1/PD-L1 axis is thus an attractive treatment direction. Lung cancer is a complex disease, and multiple mutations make it challenging to treat. Springer Nature. However, there did not appear to be a correlation between PD-L1 expression on lymphoma cells and response to treatment [33, 88, 89]. However, no randomized trial has been conducted to examine the role of allogeneic HSCT in NK/T-cell lymphomas. Am J Surg Pathol. Extranodal NK-/T-cell lymphoma usually begins in the area around the nose. 2011;118(18):491929. Blood. Bone Marrow Transpl. Liu W, Zhao D, Liu T, Niu T, Song Y, Xu W, et al. The cells stained positive for CD3 and CD56 but negative for CD20 (fig 3B3B).). T-Cell Lymphoma - StatPearls - NCBI Bookshelf 2015;47(9):10616. Article Extranodal natural killer (NK)/T cell lymphomas, nasal type, are highly aggressive non-Hodgkin lymphomas; pathologically, they present as significant vascular damage and destruction, with apparent tissue necrosis and cytotoxicity. Only one of the 11 cases reported hilar adenopathy.3 In our case, hilar and mediastinal adenopathy was not detected by CT scan or PET scan but was present at post mortem examination. . Background: To report a rare case of nasal natural killer/T (NK/T) cell lymphoma with bilateral intraocular and lung metastasis and to further describe the clinical features of intraocular manifestations. Bilateral intraocular and lung involvements associated with Nasal NK/T Prognosis is variable, with long term survival ranging from 20% to 80%. A clinically non-nasal case shown on PET/CT to have occult nasal involvement should be re-classified as a disseminated nasal one. Janus kinase 3-activating mutations identified in natural killer/T-cell lymphoma. Five patients had a durable response after a median of eighteen cycles of treatment. 2006;24(4):6128. Xiong J, Wang N, Zhong HJ, Cui BW, Cheng S, Sun R, et al. 2023 BioMed Central Ltd unless otherwise stated. National Library of Medicine Lymphoma | Johns Hopkins Kimmel Cancer Center PTCL-NOS is a subgroup of non-Hodgkin lymphoma (NHL) from post-thymic cells (mature peripheral T-cells), which accounts for ~35% of all T-cell NHLs in the North America 1,2. None of these regimens have been compared, so that their relative efficacies are undefined. Ann Oncol. Post mortem examination showed multiple masses in both lungs, hilar and mediastinal lymphadenopathy. See Lymphoma of the Skin. Yang Y, Zhu Y, Cao JZ, Zhang YJ, Xu LM, Yuan ZY, et al. These disseminate cases are referred to clinically as the aggressive lymphoma/leukaemia subtype [2, 5] and pathologically as aggressive NK-cell leukaemia by the WHO classification [9]. Lim SH, Hong JY, Lim ST, Hong H, Arnoud J, Zhao W, et al. Typical radiologic features include pulmonary nodules, consolidation, solid pulmonary opacities, cystic changes, hilar adenopathy, and pleural effusions. Immunophenotyping using immunohistochemistry or the much more sensitive flow cytometry is valuable in defining the lineage of origin. The problems of these models are the unclear and subjective definition of lymphoma local invasion, and the lack of the use of PET/CT in patient evaluation, thereby making classification of non-nasal cases insecure. GELA and GOELAMS intergroup. A computed tomographic (CT) scan of the chest showed bilateral areas of consolidation with cavitation in the right upper lobe and diffusely distributed small nodules. Exome sequencing identifies somatic mutations of DDX3X in natural killer/T-cell lymphoma. Jaccard A, Gachard N, Marin B, Rogez S, Audrain M, Suarez F, et al. In stage I/II disease, the use of radiotherapy had led to better results and survival [51, 52]. Thoracentesis was performed which also revealed a lymphocytic exudate. A central component of these regimens is asparaginase, which induces apoptosis of NK-cells in vitro [49]. To our knowledge, we report the first case of nonHIV related NK/T cell lymphoma with primary lung involvement. T-cell and NK-cell lymphomas in the lung - ScienceDirect 2016;7(23):3541222. CT of nasal-type T/NK cell lymphoma in the lung. J Hematol Oncol. The use of avelumab, an anti-PD-L1 antibody, had been studied in a prospective phase II study.93 The ORR was 38% with a CR rate of 24%. . The PET scan revealed an isolated intense hypermetabolic uptake in the right middle and lower lung regions without other identifiable adenopathy. 6 Altmetric Metrics Abstract Background Intravascular lymphoma is a rare type of non-Hodgkin lymphoma mostly of B-cell lineage. Asia Lymphoma Study Group. 2017;123(16):317685. The chest radiograph showed multiple areas of consolidation in the right lung. Survivals were very good, with 5-year PFS ranging from 64 to 83% (Table 2). Autologous EBV-specific T cell treatment results in sustained responses in patients with advanced extranodal NK/T lymphoma: results of a multicenter study. Kwong YL, Chan TSY, Tan D, Kim SJ, Poon LM, Mow B, et al. Blood. Allogeneic HSCT should be considered, although results remain anecdotal and data on HSC source and the optimal conditioning regimens are scarce. Oncol Lett. With RNA-seq and immunohistochemical studies, the TSIM, MB, and HEA subtypes were typified by overexpression of PD-L1, MYC, and DAXX, respectively. Researchers uncover potential novel therapeutic targets against natural
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