• Title, Summary, Keyword: Extranodal natural killer/T cell lymphoma

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Single Pleural Relapse of a Nasal-Type Extranodal Natural Killer/T-Cell Lymphoma: A Case Report

  • Kim, Keunmo;Oh, Youngmin;Lim, Sung-Nam;Choi, Song-Yi;Lee, Ok-Jun;Choe, Kang-Hyeon;Lee, Ki-Man;An, Jin-Young
    • Tuberculosis and Respiratory Diseases
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    • v.76 no.4
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    • pp.184-187
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    • 2014
  • A nasal-type extranodal natural killer/T-cell lymphoma is considered an aggressive form of non-Hodgkin's lymphoma, with approximately half of all patients relapsing during the follow-up period, and most relapses occurring within the first 2 years of remission. Here we report an unusual case of a 42-year-old man who experienced recurrence in single pleura after 8 years of remission.

Meta Analysis of Treatment for Stage IE~IIE Extranodal Natural Killer /T Cell Lymphomas in China

  • Li, Hui;Wang, Chun-Sen;Wang, Xiao-Dong
    • Asian Pacific Journal of Cancer Prevention
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    • v.15 no.5
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    • pp.2297-2302
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    • 2014
  • Objective: To evaluate early treatment for extranodal natural killer/T cell lymphoma (ENK/TCL) in China and provide reference for clinical treatment of these patients. Methods: Computer-based retrieval was performed in PubMed, CNKI, CBM, VIP and WanFang Data to search for randomized controlled trials (RCTs) of treatment for early ENK/TCL, and a meta-analysis was conducted with RevMan 5.0 software. Results: A total of 11 RCTs, including 871 patients, were selected, of which the first radiotherapy had a higher complete response (CR) than the first chemotherapy [OR=14.16, 95%CI (8.68, 23.10), P<0.00001] and CR was not different between combined treatment group and radiotherapy group [OR=1.86, 95%CI (0.47, 3.58), P=0.61], but long-term survival rate was higher with combined treatment[OR=1.88, 95%CI (1.09, 3.19), P=0.02]. No difference in survival rate was observed between radio-chemotherapy and chemo-radiotherapy groups [OR=1.11, 95%CI (0.73, 1.69), P=0.63]. Conclusions: Radiotherapy is of great significance in the treatment of early ENK/TCL, but combined therapy could further enhance long-term survival rate of patients. This conclusion still requires further confirmation using RCTs with high quality and large sample size.

Recurrent Extranodal NK/T-Cell Lymphoma Presenting as a Perforating Palatal Ulcer and Oro-Nasal Fistula

  • Park, Kang Gyun;Dhong, Eun Sang;Goong, Sik Nam;Han, Jung Kyu;Han, Seung Kyu;Kim, Woo Kyung
    • Archives of Craniofacial Surgery
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    • v.17 no.3
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    • pp.165-168
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    • 2016
  • Nasal-type extranodal natural killer/T-cell lymphoma (ENKTL) is a rare disease presenting with non-specific symptoms, typically originating in the nasal cavity, palate, or midfacial region. Oral cavity is an extremely rare site for this type of lymphoma. In this report, we present a case of palatal perforation and oro-nasal fistula as a manifestation of recurrent ENKTL. Complicated disease entity should be considered when surgeons deal with palatal perforation and oro-nasal fistula.

Primary Pulmonary Extranodal Natural Killer/T-cell Lymphoma, Nasal Type Presenting as Diffuse Ground Glass Opacities: a Case Report

  • Song, MyungJin;Kim, Ji-Ye;Choi, Ji Soo;Yoon, Bora;Kim, MooHyun;Kim, Soo-Jeong;Kim, Song Yee
    • Journal of Korean Medical Science
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    • v.32 no.10
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    • pp.1727-1730
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    • 2017
  • 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. However, when the corticosteroid was tapered, his symptoms recurred. The patient underwent a surgical lung biopsy and ENKTCL was diagnosed. We present this case because ENKTCL involving only the lung is very rare but very informative. To our knowledge, our patient is the first case that primary pulmonary ENKTCL is presented with GGOs.

"Sandwich" Chemotherapy (CT) with Radiotherapy (RT) Improves Outcomes in Patients with Stage IE/IIE Extranodal Natural Killer (NK)/T-cell Lymphomas

  • Zhang, Jing;Zhu, Meng-Yuan;Wang, Liang;Wang, Hua;Wang, Wei-Da;Geng, Qi-Rong;Lu, Yue
    • Asian Pacific Journal of Cancer Prevention
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    • v.14 no.7
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    • pp.4061-4066
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    • 2013
  • The extranodal natural killer/T-cell lymphoma (ENKTL) shows high local or systemic failure rates when radiotherapy (RT) is taken as the primary treatment, suggesting a role for chemotherapy (CT) added to RT for this disease. However, the appropriate mode of combined modality therapy (CMT) has not been fully defined. A total of one hundred and twenty-one patients with ENKTL receiving sandwich CT with RT were reviewed between January 2003 and August 2012. The primary endpoints were the response rate, progression-free survival (PFS), overall survival (OS), and the relapse rate. After the initial CT, there were 84 (69.4%) patients in CR, 22 (18.2%) patients in PR, 9 (7.4%) patients in SD, and 6 (5%) patients in PD, respectively. At the end of RT, the CR, PR, SD, and PD rates for all patients were 90.9% (n=110), 1.7% (n=2), 4.1% (n=5), and 3.3% (n=4), respectively. After a median follow-up of 42.3 months (3.5~112.3 months), the 5-year PFS was 74.7% (95% CI 70.4%~79.0%), and 5-year OS was 77.3% (95% CI 67.9%~86.7%). Disease progression was documented in 25 (20.7%) patients. The rates of systemic failure, local failure, and regional failure were 18.2%, 5.8%, 1.7%, respectively. Twenty death events (16.5%) were observed for the entire group of patients (18 deaths related to PD). Furthermore, CR to the initial CT and low Korean Prognostic Index (KPI) can independently predict long PFS and OS. The sandwich CMT achieved an excellent outcome for localized ENKTL with acceptable toxicity. We recommend it can be applied as the optimal choice for localized ENKTL.

Extranodal NK/T cell Lymphoma, nasal type: clinical, radiological, histological features for early diagnosis (원발성 비성 NK/T 세포 림프종: 조기 진단을 위한 임상적, 방사선학적, 조직학적 특징)

  • Park, Kyung-Ran;Han, Seon-Hee;Kim, Hyun-Sil;Lee, Seung-Jun;Cha, In-Ho;Kim, Hyung-Jun
    • Journal of the Korean Association of Oral and Maxillofacial Surgeons
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    • v.36 no.6
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    • pp.497-501
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    • 2010
  • Primary nasal type natural killer (NK)/T cell (NKTC) lymphoma, a specific form of malignant lymphoma, has a higher geographic incidence in Oriental, Mexican, and South American populations than the Western population. In Koreans, it comprises 9-12% of all cases of non-Hodgkin's lymphoma. This type of lymphoma has also been named as angiocentic lymphoma and lethal midline granuloma because the most common site is the upper airway area and its clinical aggressiveness presents with a necrotic and destructive pattern. NKTC lymphoma can also be detected in different organs (testis, spleen, parotid gland, skin, gastroinstinal tract, central nervous system, lungs, bone marrow, etc.) other than the upper airway including the oral cavity. The lymphoma detected in the oral cavity shows various destructive and inflammatory changes, similar to the signs of inflammation and infection from periodontitis and pulpal disease, making a diagnosis difficult with just the clinical signs. For early detection, clinical, radiological, and pathological examinations are required. This report describes the clinical, radiological and histological characteristics with a case report for the early detection of NKTC lymphoma in the oral cavity.

Mutational Analysis of Extranodal NK/T-Cell Lymphoma Using Targeted Sequencing with a Comprehensive Cancer Panel

  • Choi, Seungkyu;Go, Jai Hyang;Kim, Eun Kyung;Lee, Hojung;Lee, Won Mi;Cho, Chun-Sung;Han, Kyudong
    • Genomics & Informatics
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    • v.14 no.3
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    • pp.78-84
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    • 2016
  • Extranodal natural killer (NK)/T-cell lymphoma, nasal type (NKTCL), is a malignant disorder of cytotoxic lymphocytes of NK or T cells. It is an aggressive neoplasm with a very poor prognosis. Although extranodal NKTCL reportedly has a strong association with Epstein-Barr virus, the molecular pathogenesis of NKTCL has been unexplored. The recent technological advancements in next-generation sequencing (NGS) have made DNA sequencing cost- and time-effective, with more reliable results. Using the Ion Proton Comprehensive Cancer Panel, we sequenced 409 cancer-related genes to identify somatic mutations in five NKTCL tissue samples. The sequencing analysis detected 25 mutations in 21 genes. Among them, KMT2D, a histone modification-related gene, was the most frequently mutated gene (four of the five cases). This result was consistent with recent NGS studies that have suggested KMT2D as a novel driver gene in NKTCL. Mutations were also found in ARID1A, a chromatin remodeling gene, and TP53, which also recurred in recent NGS studies. We also found mutations in 18 novel candidate genes, with molecular functions that were potentially implicated in cancer development. We suggest that these genes may result in multiple oncogenic events and may be used as potential bio-markers of NKTCL in the future.

Efficacy and Tolerance of Pegaspargase-Based Chemotherapy in Patients with Nasal-Type Extranodal NK/T-Cell Lymphoma: a Pilot Study

  • Wen, Jing-Yun;Li, Mai;Li, Xing;Chen, Jie;Lin, Qu;Ma, Xiao-Kun;Dong, Min;Wei, Li;Chen, Zhan-Hong;Wu, Xiang-Yuan
    • Asian Pacific Journal of Cancer Prevention
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    • v.15 no.15
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    • pp.6275-6281
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    • 2014
  • Nasal-type extranodal natural killer (NK)/T-cell lymphoma (ENKL) is a highly invasive cancer with a poor prognosis. More effective and safer treatment regimens for ENKL are needed. Pegaspargase (PEG-Asp) has a similar mechanism of action to L-asparaginase (L-Asp), but presents lower antigenicity. The aim of the present research was to evaluate the safety profile and the latent efficacy of a PEG-Asp-based treatment regimen in patients with ENKL. Data collected from 20 patients with histologically confirmed ENKL, admitted to the Third Affiliated Hospital of Sun Yat-Sen University from January 2009 to August 2013, were included in the study. All patients received $2500IU/m^2$/IM PEG-Asp on day 1 of every 21-day treatment cycle. Patients received combination chemotherapy with CHOP (n=5), EPOCH (n=7), GEMOX (n=7) or CHOP with bleomycin (n=1). After 2-5 treatment cycles (median, 4 cycles) of PEG-Asp-based chemotherapy, five patients (25%) showed a complete response (CR), and the overall response rate (ORR) was 60%. Grade 3/4 neutropenia occurred in fourteen patients (70%). Grade 3 alanine aminotransferase (ALT) elevation was observed in two. Grade 1-2 non-hematological toxicity consisted of activated partial thromboplastin time (APTT) elongation (n=9), hypofibrinogenemia (n=6), hypoproteinemia (n=17), hyperglycemia (n=3), and nausea (n=6). No allergic reactions were detected. No treatment related death was reported. Our results suggested that PEG-Asp-based chemotherapy presented an acceptable tolerance and a potential short-term outcome in patients with nasal-type ENKL.