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1. Kim TH, Kim JS, Suh YG, Cho J, Yang WI, Suh CO: The Roles of Radiotherapy and Chemotherapy in the Era of Multimodal Treatment for Early-Stage Nasal-Type Extranodal Natural Killer/T-Cell Lymphoma. Yonsei Med J; 2016 Jul;57(4):846-54
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  • [Title] The Roles of Radiotherapy and Chemotherapy in the Era of Multimodal Treatment for Early-Stage Nasal-Type Extranodal Natural Killer/T-Cell Lymphoma.
  • PURPOSE: To evaluate radiotherapy (RT) and chemotherapy (CT) treatments of early-stage extranodal natural killer/T-cell lymphoma (ENKTL).
  • [MeSH-major] Chemoradiotherapy. Lymphoma, Extranodal NK-T-Cell / drug therapy. Lymphoma, Extranodal NK-T-Cell / radiotherapy

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  • (PMID = 27189276.001).
  • [ISSN] 1976-2437
  • [Journal-full-title] Yonsei medical journal
  • [ISO-abbreviation] Yonsei Med. J.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Korea (South)
  • [Other-IDs] NLM/ PMC4951459
  • [Keywords] NOTNLM ; Lymphoma / radiotherapy / recurrencec
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2. Haverkos BM, Pan Z, Gru AA, Freud AG, Rabinovitch R, Xu-Welliver M, Otto B, Barrionuevo C, Baiocchi RA, Rochford R, Porcu P: Extranodal NK/T Cell Lymphoma, Nasal Type (ENKTL-NT): An Update on Epidemiology, Clinical Presentation, and Natural History in North American and European Cases. Curr Hematol Malig Rep; 2016 Dec;11(6):514-527
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  • [Title] Extranodal NK/T Cell Lymphoma, Nasal Type (ENKTL-NT): An Update on Epidemiology, Clinical Presentation, and Natural History in North American and European Cases.
  • PURPOSE OF REVIEW: Extranodal NK/T cell lymphoma, nasal type (ENKTL-NT) is an aggressive extranodal non-Hodgkin lymphoma most commonly occurring in East Asia and Latin America but with increasing incidence in the United States.
  • RECENT FINDINGS: We highlight key observations in its epidemiology, natural history, and trends in clinical management.
  • While there is variation in age at diagnosis, presence of antecedent lymphoproliferative disorders, and outcomes among racial/ethnic groups, the universal association of ENKTL-NT with EBV and the poor response of this neoplasm to anthracycline-based therapy is consistent across all geographic areas.
  • Data on epidemiology, disease presentation, and clinical outcomes in mature T cell and NK cell (T/NK cell) neoplasms, including ENKTL-NT, in Europe and North America are very limited.
  • As the classification and diagnostic characterization of the currently recognized T/NK cell lymphoma disease entities continue to evolve, gaps and inconsistencies in data reporting across different studies are being recognized.
  • Given the overlap between the entities included in the category of T/NK cell neoplasms, there is a need to further define biological and clinical differences that may affect diagnosis, treatment, and outcome.
  • [MeSH-major] Lymphoma, Extranodal NK-T-Cell / pathology

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  • (PMID = 27778143.001).
  • [ISSN] 1558-822X
  • [Journal-full-title] Current hematologic malignancy reports
  • [ISO-abbreviation] Curr Hematol Malig Rep
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / L30 CA189070
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Biomarkers; 0 / DNA, Viral; EC 3.5.1.1 / Asparaginase
  • [Keywords] NOTNLM ; ENKTL / Epstein–Barr virus / Europe / Extranodal NK/T cell lymphoma / United Kingdom / United States
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3. Yang QP, Zhang WY, Yu JB, Zhao S, Xu H, Wang WY, Bi CF, Zuo Z, Wang XQ, Huang J, Dai L, Liu WP: Subtype distribution of lymphomas in Southwest China: analysis of 6,382 cases using WHO classification in a single institution. Diagn Pathol; 2011;6:77
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  • [Title] Subtype distribution of lymphomas in Southwest China: analysis of 6,382 cases using WHO classification in a single institution.
  • This study aims to analyze subtype distribution of lymphomas in southwest China.
  • RESULTS: From January 2000 to December 2008, a total number of 6,382 patients with lymphoma were established, of which mature B-cell neoplasms accounted for 56%, mature T- and NK-cell neoplasms occupied 26%, and precursor lymphoid neoplasms and Hodgkin lymphomas were 5% and 13%, respectively.
  • Mixed cellularity (76%) was the major subtype of classical Hodgkin lymphoma; and the bimodal age distribution was not observed.
  • The top six subtypes of non-Hodgkin lymphoma were as follows: diffuse large B-cell lymphoma, extranodal NK/T-cell lymphoma, nasal type, extranodal marginal zone lymphoma of mucosa associated lymphoid tissue, follicular lymphoma, precursor lymphoid neoplasms, and chronic lymphocytic leukemia/small lymphocytic lymphoma.
  • Extranodal lymphomas comprised about half of all cases, and most frequently involved Waldeyer's ring, gastrointestinal tract, sinonasal region and skin.
  • The high frequency of mature T/NK cell neoplasms and extranodal lymphomas, especially for extranodal NK/T-cell lymphoma, nasal type, is the most outstanding characteristic of this series.
  • [MeSH-major] Lymphoma / diagnosis. World Health Organization

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  • (PMID = 21854649.001).
  • [ISSN] 1746-1596
  • [Journal-full-title] Diagnostic pathology
  • [ISO-abbreviation] Diagn Pathol
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC3179701
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4. Baran AM, Reifenberger J, Helbig D: [NK/T-cell lymphoma, nasal type with cutaneous dissemination]. Hautarzt; 2012 Apr;63(4):272-5
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  • [Title] [NK/T-cell lymphoma, nasal type with cutaneous dissemination].
  • [Transliterated title] NK/T-Zell-Lymphom vom nasalen Typ mit kutaner Dissemination.
  • Extranodal NK/T-cell lymphoma, nasal type, is a lymphoproliferative disorder originating from peripheral T-cells or natural killer (NK) cells.
  • Due to nonspecific histological and clinical findings mimicking a chronic inflammatory condition, a diagnosis of Wegener disease was made and immunosuppressive therapy with azathioprine was initiated.
  • Histopathological reevaluation and immunophenotyping revealed a disseminated NK/T-cell lymphoma, nasal type.
  • In the case of an unspecific chronic inflammatory process in the nasopharyngeal space one should always consider the possibility of this rare lymphoma, even in Europe.
  • [MeSH-major] Lymphoma, Extranodal NK-T-Cell / pathology. Neoplasms, Multiple Primary / pathology. Nose Neoplasms / pathology. Skin Neoplasms / pathology
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Cyclophosphamide / administration & dosage. Diagnosis, Differential. Doxorubicin / administration & dosage. Female. Humans. Middle Aged. Prednisone / administration & dosage. Treatment Outcome. Vincristine / administration & dosage

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  • (PMID = 22430611.001).
  • [ISSN] 1432-1173
  • [Journal-full-title] Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete
  • [ISO-abbreviation] Hautarzt
  • [Language] ger
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; VB0R961HZT / Prednisone; CHOP protocol
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5. Liang L, Nong L, Zhang S, Zhao J, Ti H, Dong Y, Zhang B, Li T: The downregulation of PRDM1/Blimp-1 is associated with aberrant expression of miR-223 in extranodal NK/T-cell lymphoma, nasal type. J Exp Clin Cancer Res; 2014;33:7
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  • [Title] The downregulation of PRDM1/Blimp-1 is associated with aberrant expression of miR-223 in extranodal NK/T-cell lymphoma, nasal type.
  • BACKGROUND: The mechanism for inactivation of positive regulatory domain containing I (PRDM1), a newly identified tumour suppressor gene in extranodal NK/T-cell lymphoma, nasal type (EN-NK/T-NT) has not been well defined.
  • The aim of the present study was to investigate the expression of PRDM1 in EN-NK/T-NT and analyse its downregulation by miRNAs.
  • METHODS: PRDM1 and miRNA expression were evaluated in EN-NK/T-NT samples by immunohistochemical analysis, qRT-PCR, and in situ hybridisation.
  • In addition, the effect of miR-223 on PRDM1 expression was assessed in NK/T lymphoma cell lines by transfecting a miR-223 mimic or inhibitor to increase or decrease the effective expression of miR-223.
  • Overall survival and failure-free survival in EN-NK/T-NT patients were analysed using Kaplan-Meier single-factor analysis and the log-rank test.
  • RESULTS: Investigation of the downregulation of PRDM1 in EN-NK/T-NT cases revealed that PRDM1-positive staining might be a favourable predictor of overall survival and failure-free survival in EN-NK/T-NT patients.
  • However, the negative staining of PRDM1 usually presented transcripts, suggesting a possible post-transcriptional regulation. miR-223 and its putative target gene, PRDM1, exhibited opposite patterns of expression in EN-NK/T-NT tissues and cell lines.
  • The ectopic expression of miR-223 led to the downregulation of the PRDM1 protein in the NK/T-cell lymphoma cell line, whereas a decrease in miR-223 restored the level of PRDM1 protein.
  • CONCLUSIONS: Our findings reveal that the downregulation of the tumour suppressor PRDM1 in EN-NK/T-NT samples is mediated by miR-223 and that PRDM1-positive staining might have prognostic value for evaluating the clinical outcome of EN-NK/T-NT patients.
  • [MeSH-major] Lymphoma, Extranodal NK-T-Cell / metabolism. MicroRNAs / genetics. Nose Neoplasms / metabolism. Repressor Proteins / genetics
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Base Sequence. Binding Sites. Cell Line, Tumor. Child. Disease-Free Survival. Down-Regulation. Female. Gene Expression. Gene Expression Regulation, Neoplastic. Humans. Kaplan-Meier Estimate. Male. Middle Aged. RNA Interference. Retrospective Studies. Young Adult

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  • (PMID = 24438193.001).
  • [ISSN] 1756-9966
  • [Journal-full-title] Journal of experimental & clinical cancer research : CR
  • [ISO-abbreviation] J. Exp. Clin. Cancer Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / MIRN223 microRNA, human; 0 / MicroRNAs; 0 / Repressor Proteins; 138415-26-6 / PRDM1 protein, human
  • [Other-IDs] NLM/ PMC3898819
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6. Lee S, Park HY, Kang SY, Kim SJ, Hwang J, Lee S, Kwak SH, Park KS, Yoo HY, Kim WS, Kim JI, Ko YH: Genetic alterations of JAK/STAT cascade and histone modification in extranodal NK/T-cell lymphoma nasal type. Oncotarget; 2015 Jul 10;6(19):17764-76
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  • [Title] Genetic alterations of JAK/STAT cascade and histone modification in extranodal NK/T-cell lymphoma nasal type.
  • Extranodal NK/T-cell lymphoma nasal type (ENKL) is a rare type of non-Hodgkin lymphoma that more frequently occurs in East Asia and Latin America.
  • Here, we performed multiple types of next-generation sequencing on 34 ENKL samples, including whole-exome sequencing (9 cancer tissues and 4 cancer cell lines), targeted sequencing (21 cancer tissues), and RNA sequencing (3 cancer tissues and 4 cancer cell lines).
  • In addition, we provided 177 genes upregulated only in cancer tissues, which appear to be linked with angiocentric and angiodestructive growth of ENKL.
  • [MeSH-major] Histones / genetics. Janus Kinases / genetics. Lymphoma, Extranodal NK-T-Cell / genetics. STAT Transcription Factors / genetics

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  • (PMID = 25980440.001).
  • [ISSN] 1949-2553
  • [Journal-full-title] Oncotarget
  • [ISO-abbreviation] Oncotarget
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Histones; 0 / STAT Transcription Factors; EC 2.7.10.2 / Janus Kinases
  • [Other-IDs] NLM/ PMC4627344
  • [Keywords] NOTNLM ; JAK-STAT pathway / chromatin modification / extranodal NK/T-cell lymphoma nasal type / next-generation sequencing / somatic mutation
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7. Tse E, Kwong YL: The diagnosis and management of NK/T-cell lymphomas. J Hematol Oncol; 2017 04 14;10(1):85
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The diagnosis and management of NK/T-cell lymphomas.
  • 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.
  • Pathologically, the malignancy occurs in two forms, extranodal NK/T-cell lymphoma, nasal type; and aggressive NK-cell leukaemia.
  • Lymphoma occur most commonly (80%) in the nose and upper aerodigestive tract, less commonly (20%) in non-nasal areas (skin, gastrointestinal tract, testis, salivary gland), and rarely as disseminated disease with a leukemic phase.
  • In the era of effective non-anthracycline-containing regimens, autologous haematopoietic stem cell transplantation (HSCT) has not been shown to be beneficial.
  • [MeSH-major] Lymphoma, Extranodal NK-T-Cell / diagnosis. Lymphoma, Extranodal NK-T-Cell / therapy
  • [MeSH-minor] Asparaginase / therapeutic use. Combined Modality Therapy / methods. Hematopoietic Stem Cell Transplantation. Humans. Prognosis. Programmed Cell Death 1 Receptor / antagonists & inhibitors. Radiotherapy. Salvage Therapy / methods

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  • (PMID = 28410601.001).
  • [ISSN] 1756-8722
  • [Journal-full-title] Journal of hematology & oncology
  • [ISO-abbreviation] J Hematol Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Review; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / PDCD1 protein, human; 0 / Programmed Cell Death 1 Receptor; EC 3.5.1.1 / Asparaginase
  • [Keywords] NOTNLM ; EBV DNA quantification (major topic) / Extranodal (major topic) / Immunotherapy (major topic) / L-asparaginase (major topic) / NK/T-cell lymphoma (major topic) / Nasal (major topic) / Non-nasal (major topic) / PD1 (major topic) / Prognostication (major topic)
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8. Wang X, Gong Z, Li SX, Yan W, Song Y: Extranodal nasal-type natural killer/T-cell lymphoma with penile involvement: a case report and review of the literature. BMC Urol; 2017 Sep 06;17(1):77
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  • [Title] Extranodal nasal-type natural killer/T-cell lymphoma with penile involvement: a case report and review of the literature.
  • BACKGROUND: Extranodal natural killer/T-cell lymphoma (ENKTL) usually presents as a localized disease in the nasal cavity; extension to the male genitourinary system is very rare and has been characterized only recently.
  • Most cases present with predominantly extranodal involvement, advanced stage disease, highly aggressive course, and strong association with Epstein-Barr virus (EBV).
  • While metastasis is common in ENKTLs, the penis is rarely involved in both nasal and non-nasal ENKTLs and only one report was published to date.
  • CASE PRESENTATION: One patient with NK/T-cell lymphoma, presented initially with a penile mass, is reported.
  • Histologically, the glans and foreskin revealed neoplastic infiltration of medium-sized lymphoma cells expressing CD56, CD3, granzyme-B, and labeled for EBV-encoded RNA in situ hybridization.
  • Findings were consistent with NK/T-cell lymphoma.
  • By detailed history, we learned that the patient had nasal obstruction for more than 10 years.
  • The final diagnosis was primary nasal NK/T-cell lymphoma, with metastasis to the penis.
  • CONCLUSION: This study suggested that penile NK/T-cell lymphoma tends to disseminate early and pursues an aggressive course.
  • It is imperative to distinguish nasal NK/T lymphoma from other types of tumors, because the prognosis and treatment differ significantly for secondary metastases.

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  • (PMID = 28874193.001).
  • [ISSN] 1471-2490
  • [Journal-full-title] BMC urology
  • [ISO-abbreviation] BMC Urol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Keywords] NOTNLM ; NK/T lymphoma / Penile malignancy
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9. Suzuki R: Treatment of advanced extranodal NK/T cell lymphoma, nasal-type and aggressive NK-cell leukemia. Int J Hematol; 2010 Dec;92(5):697-701
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Treatment of advanced extranodal NK/T cell lymphoma, nasal-type and aggressive NK-cell leukemia.
  • Extranodal NK/T cell lymphoma, nasal type (ENKL) with advanced stage and aggressive NK-cell leukemia (ANKL) are highly aggressive neoplasms with a dismal clinical outcome.
  • This is a major reason for the refractoriness to conventional chemotherapeutic regimens for malignant lymphoma containing anthracycline.
  • ANKL needs another treatment strategy because of a systemic disease progression and extensive organ insufficiency.
  • Optimal treatment scheme using such effective agents for these unfavorable NK-cell tumors should further be explored.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Killer Cells, Natural / pathology. Leukemia, T-Cell / therapy. Lymphoma, Extranodal NK-T-Cell / drug therapy

  • Genetic Alliance. consumer health - Aggressive NK Cell Leukemia.
  • Hazardous Substances Data Bank. ETOPOSIDE .
  • Hazardous Substances Data Bank. IFOSFAMIDE .
  • Hazardous Substances Data Bank. DEXAMETHASONE .
  • Hazardous Substances Data Bank. METHOTREXATE .
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  • (PMID = 21116747.001).
  • [ISSN] 1865-3774
  • [Journal-full-title] International journal of hematology
  • [ISO-abbreviation] Int. J. Hematol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 6PLQ3CP4P3 / Etoposide; 7S5I7G3JQL / Dexamethasone; EC 3.5.1.1 / Asparaginase; UM20QQM95Y / Ifosfamide; YL5FZ2Y5U1 / Methotrexate
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10. Tan B, Abdelmalek C, O'Donnell JE, Toltaku T, Chaudhry R, Wang JC, Gotlieb V: A Case Report of Primary Nasal Natural Killer (NK)/T-Cell Lymphoma in an African American Patient Presenting with Hemophagocytic Syndrome. Am J Case Rep; 2017 Feb 14;18:160-165
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A Case Report of Primary Nasal Natural Killer (NK)/T-Cell Lymphoma in an African American Patient Presenting with Hemophagocytic Syndrome.
  • BACKGROUND Extranodal natural killer/T-cell lymphoma, nasal type (ENKTCL) is generally an aggressive and rare non-Hodgkin lymphoma.
  • CASE REPORT A 55-year-old African American male born in Grenada presented with a left nostril mass with facial swelling and biopsy subsequently confirmed a diagnosis of extranodal NK/T-cell lymphoma, nasal type (ENKTCL).
  • Bone marrow aspiration did not show lymphoma involvement.
  • We reached a diagnosis of hemophagocytic syndrome.
  • CONCLUSIONS To the best of our knowledge, this is the only second report of extranodal NK/T-cell lymphoma, nasal type in a black patient, and it raises the awareness of early recognition of rare manifestations of NK/T-cell lymphoma such as hemophagocytic syndrome.
  • [MeSH-major] African Americans. Lymphohistiocytosis, Hemophagocytic / diagnosis. Lymphoma, Extranodal NK-T-Cell / diagnosis

  • MedlinePlus Health Information. consumer health - African American Health.
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  • [ISSN] 1941-5923
  • [Journal-full-title] The American journal of case reports
  • [ISO-abbreviation] Am J Case Rep
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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