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[Analysis of the clinical diagnosis and treatment of extranodal NK-T cell lymphoma nasal type]. Li H X,Zhang L R,Zhang L L Lin chuang er bi yan hou tou jing wai ke za zhi = Journal of clinical otorhinolaryngology, head, and neck surgery 10.13201/j.issn.1001-1781.2018.13.018
The diagnosis and management of NK/T-cell lymphomas. Journal of hematology & oncology 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. Genetic analysis showed mutations of genes involved in the JAK/STAT pathway, RNA assembly, epigenetic regulation, and tumor suppression. In initial clinical evaluation, positron emission tomography computed tomography, and quantification of plasma EBV DNA are mandatory as they are useful for response monitoring and prognostication. In stage I/II diseases, combined chemotherapy and radiotherapy (sequentially or concurrently) is the best approach. Conventional anthracycline-containing regimens are ineffective and should be replaced by non-anthracycline-containing regimens, preferably including L-asparaginase. Radiotherapy alone is associated with high systemic relapse rates and should be avoided. In stage III/IV diseases, non-anthracycline-regimens-containing L-asparaginase are the standard. In relapsed/refractory cases, blockade of the programmed death protein 1 has recently shown promising results with high response rates. In the era of effective non-anthracycline-containing regimens, autologous haematopoietic stem cell transplantation (HSCT) has not been shown to be beneficial. However, allogeneic HSCT may be considered for high-risk or advanced-stage patients in remission or relapsed/refractory patients responding to salvage therapy. Prognostic models taking into account presentation, interim, and end-of-treatment parameters are useful in triaging patients to different treatment strategies. 10.1186/s13045-017-0452-9
A case report of extranodal NK/T-cell lymphoma in patient with chronic lymphocytic leukemia. Ge Hangping,Wu Xiangping,Shen Jianping,Chen Junfa,Chen Ying,Zhang Yu Medicine RATIONALE:Chronic lymphocytic leukemia often results in secondary tumors, the most common being large B cell lymphoma known as Richter syndrome, followed by extranodal NK/T-cell lymphoma (nasal type) is extremely rare. PATIENT CONCERNS:A chronic lymphocytic leukemia patient presented with nasal congestion. DIAGNOSES:Nasal endoscopy identified a left nasal mass, and the pathology suggested extranodal NK/T-cell lymphoma (nasal type). INTERVENTIONS:The patient received a course of chemotherapy. OUTCOMES:Pneumonia and coagulopathy occurred after chemotherapy, and the patient died shortly thereafter. LESSONS:It is essential to recognize the transformation of disease earlier in chronic lymphoblastic leukemia patient. 10.1097/MD.0000000000011619
Extranodal NK/T-cell lymphoma, nasal type of the uterine cervix: A case report. Omori Makiko,Oishi Naoki,Nakazawa Tadao,Nakazawa Kumiko,Mitsumori Toru,Yuminamochi Tsutomu,Kirito Keita,Hirata Shuji Diagnostic cytopathology We report a rare case of extranodal NK/T-cell lymphoma, nasal type of the uterine cervix that showed cytologic features mimicking cervical cancer. A 65-year-old woman presented with vaginal bleeding. Gynecological examination revealed a bulky tumor of the cervix. A conventional Papanicolaou-stained cervical smear showed hypercellularity consisting of numerous variably sized cohesive clusters that mimicked epithelial tumors, with a necrotic and inflammatory background. A small number of individually scattered cells were also identified. These scattered cells showed pleomorphic, often cleaved, or horseshoe-shaped nuclei and pale cytoplasm. Biopsy specimens revealed a diffuse growth of atypical cells with an angiocentric pattern. Extensive necrosis and infiltration of inflammatory cells were present. There were numerous mitotic figures. The tumor cells were positive for CD45RO, CD3ε, CD56, granzyme B, TIA-1, CD7, and Epstein-Barr virus (EBV)-encoded small RNA (EBER) by in situ hybridization, and negative for cytokeratin, chromogranin A, synaptophysin, CD4, CD5, CD8, CD20, and CD30. Based on these findings, this tumor was diagnosed as extranodal NK/T-cell lymphoma, nasal type of the uterine cervix. 10.1002/dc.23439
Comparative Study of L-Asparaginase-Based LOP Regimen Over CHOP Regimen Before Radiotherapy for Stage IIE Extranodal Nasal Type NK/T Cell Lymphoma: A Study of 2 Centers. Huang Limin,Yuan Bin,Wu Haixia,Chu Hongliang,Liu Yayun,Wu Shuang,Li Hong,Lu He,Chen Hui Clinical lymphoma, myeloma & leukemia BACKGROUND:In this study we evaluated the efficacy of an L-asparaginase-based LOP (L-asparaginase, vincristine, and dexamethasone) regimen in extranodal Natural Killer (NK)/T-cell lymphoma (ENKTL) patients in the Guizhou province of China. PATIENTS AND METHODS:Forty-eight patients were treated with the LOP (L-asparaginase, vincristine and dexamethasone) regimen chemotherapy (CT) and 32 patients with the CHOP (cyclophosphamide, tetrahydropyanyl adriamycin, vincristine, and prednisone) regimen. These patients then received involved-field radiotherapy (RT) with the doses of DT = 49-59 Gy. RESULTS:A significant improvement of clinical end points with the LOP regimen was noticed compared with the CHOP regimen: 33 (68.8%) versus 16 (50.0%) for complete responses; 10 (20.8%) versus 5 (15.6%) for partial responses. There were statistical differences in objective response rates (43 [89.6%] for LOP vs. 21 [65.6%] for CHOP; P = .009), 3 years of overall survival (42 [87.5%] for LOP vs. 20 [62.5%] for CHOP; P = .006) and progression-free survival (32 [79.2%] for LOP vs. 16 [50.0%] for CHOP; P = .007). CONCLUSION:The results showed that the LOP regimen is safe and much more efficient than the CHOP regimen for stage IIE ENKTL patients. They indicate that the LOP regimen is a satisfying alternative protocol among the other L-asparaginase-based regimens reported so far, such as SMILE (dexamethasone, methotrexate, ifosfamide, L-asparaginase, and etoposide), GELOX (gemcitabine, oxaliplatin, and L-asparaginase), CHOP-L, and sandwich (CT, then RT, then CT). 10.1016/j.clml.2016.12.003
[Clinical observation of LOP chemotherapy combined with radiotherapy in the treatment of early nasal NK/T cell lymphoma]. Ai W B,Jing Q C Lin chuang er bi yan hou tou jing wai ke za zhi = Journal of clinical otorhinolaryngology, head, and neck surgery To investigate the efficacy and safety of LOP(asparaginase + vincristine + dexamethasone) chemotherapy combined with radiotherapy in patients with nasal NK/T cell lymphoma. Sixty patients with nasal NK/T cell lymphoma admitted to our hospital from February 2012 to February 2016 were selected as the study subject. They were randomly divided into group A and group B, 30 cases in each group. All patients were treated with combined chemotherapy and IMRT(intensity modulated conformal radiotherapy). The LOP regimen was used in group A and the CHOP(cyclophosphamide + pirarubicin + vincristine + dexamethasone) regimen was used in group B. The short-term efficacy, long-term efficacy and adverse reactions of the two groups were compared. The clinical manifestations of 60 patients mainly included nasal obstruction(81.67%), accompanied by fever, headache, nosebleed and runny nose. Forty-one patients(68.33%) had only one site of lesion, and 21 patients(35.00%) had multiple sites of lesions. In terms of total remission rate, it was significantly higher in group A than that in group B(93.33% vs. 66.67%, <0.05). In terms of adverse reactions, the incidence of bone marrow suppression, gastrointestinal reaction and low-protein reaction was significantly lower in group A than that in group B(<0.05). Three patients died in group A and 11 patients died in group B during the 3-year follow-up. The 3-year survival rate of group A was higher than that of group B(<0.05). Compared with CHOP+IMRT regimen, the LOP+IMRT regimen for nasal NK/T-cell lymphoma patients resulted in higher overall remission rate, survival rate and lower adverse reactions, so it is worth in clinical promotion. 10.13201/j.issn.1001-1781.2019.10.022
[A case of nasal NK/T-cell lymphoma]. Zhan Yi,Zhang Guangcheng,Liu Jian,Luo Shuangyan,Lu Qianjin,Xiao Rong,Zhang Guiying Zhong nan da xue xue bao. Yi xue ban = Journal of Central South University. Medical sciences A 29-year-old male patient with extranodal NK/T-cell lymphoma, a nasal type lymphoma with involvement of skin as the first symptom, was reported. The patient presented with swelling in the left side of the nose and suffered intermittent fever for 1 month. The fester in the oral mucosa and skin under the left nostril and redness, and the swelling on the orbit of the left eye lasted for 1 week. Physical examination showed that the left side of nose was swelling, and the skin below the left nostril was anabrotic and crusted. There were different ulcers in his jaws and buccal mucosa. Bilateral eyelid was redness and swelling, especially in the left side. Binocular conjunctival was congestive. The diagnosis of extranodal NK/T-cell lymphoma (nasal type) was confirmed by biopsy and immunohistochemistry. 10.11817/j.issn.1672-7347.2017.07.020
Extranodal NK-T Cell Lymphoma, Nasal Type: Retrospective Analysis of Real-World Data. Bothra Sneha J,Bhandari Pragya,Agrawal Narendra,Tejwani Narender,Ahmed Rayaz,Khushoo Vishvdeep,Mirgh Sumeet,Bhurani Dinesh Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion Peripheral T cell lymphomas constitute nearly 15% of all cases on non-Hodgkin lymphoma. Of these, NK-T cell lymphoma nasal type is a rare and aggressive form. We present our experience of 16 patients of NK/T cell lymphoma which constituted approximately 1% of all lymphoma (N = 1590) cases treated at our center. Male to female ratio was 4.3:1. Median age of presentation was 42 years. Early Stage patients (n = 11) were treated with DeVIC regimen (n = 10) and SMILE (n = 1) chemotherapy and RT to all the patients. Advanced stage patients were treated with SMILE regimen (n = 4) and ICE and local RT (n = 1) with one treatment related mortality. The presence of B-symptoms adversely affected survival. The estimated median PFS and OS were 39 and 49 months respectively. Overall survival was not reached in Limited Stage patients (stage 1 and 2) and 8 months in patients with advanced stage (stage IV) ( = 0.001). According to the new CSWOG staging (retrospectively applied), comparing the Limited versus Extensive Stage, the earlier group has a significantly better estimated PFS ( = 0.020) and OS ( = 0.007). ENKTL is a rare malignancy with aggressive course. B-symptoms portend a poor prognosis to patients with this aggressive lymphoma. The new staging system helps estimate survival better. 10.1007/s12288-019-01195-6
A Case Report of Primary Nasal Natural Killer (NK)/T-Cell Lymphoma in an African American Patient Presenting with Hemophagocytic Syndrome. Tan Bowei,Abdelmalek Cherif,O'Donnell James E,Toltaku Thomas,Chaudhry Rashid,Wang Jen C,Gotlieb Vladimir The American journal of case reports BACKGROUND Extranodal natural killer/T-cell lymphoma, nasal type (ENKTCL) is generally an aggressive and rare non-Hodgkin lymphoma. It is most common in East Asians, Native Americans, and South Americans, but is rarely reported in blacks. 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). Immunochemistry was positive for CD2, cytoplasmic CD3, CD7, CD 43, CD 56, granzyme B, and TIA-1. In situ hybridization was positive for Epstein-Barr virus encoded ribonucleic acid (EBERs). Bone marrow aspiration did not show lymphoma involvement. The patient had progressive neutropenia upon presentation, with further investigations showing hepatomegaly, hyperferritinemia, and hemophagocytosis in the bone marrow. We reached a diagnosis of hemophagocytic syndrome. He was treated with a high-dose combination chemotherapy and radiation therapy; the neutropenia improved significantly with steroids as treatment for immune activation in the setting 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. 10.12659/ajcr.900995
Cutaneous extranodal natural killer (NK) / T - cell lymphoma: A comprehensive clinical features and outcomes analysis of 71 cases. Jiang Li,Li Pengfei,Quan Qi,Chen Ping,Qiu Huijuan,Zhang Bei Leukemia research BACKGROUND:The skin is the common extranasal sites in extranodal natural killer (NK) / T - cell lymphoma, (ENKTL). However, data regarding cutaneous ENKTL are relatively scarce. Thus, we performed a large retrospective study to analyze clinical features and treatment outcomes of cutaneous ENKTL. PATIENTS AND METHODS:Seventy-one patients with cutaneous ENKTL newly diagnosed between 2001 and 2018 were retrospectively analyzed from Sun Yat-sen cancer center. RESULTS:The median overall survival (OS) was 15.5 months (95 % confidence interval: 8.0-22.1 months). Age, treatment strategy, and treatment response were independent prognostic factors for cutaneous ENKTL. Patients who could undergo chemotherapy followed by radiotherapy showed a significantly better survival than those treated with chemotherapy alone (p < 0.001). The 3 - year OS rates were 73.9 % for patients who achieved complete response (CR) compared with 10.3 % for patients who did not (p < 0.001). CONCLUSION:We reported by far the largest series of cutaneous ENKTL, and demonstrated its aggressive clinical behavior and unsatisfying response to the current therapeutic strategies. Further investigations are required for the better understanding and management of this uncommon disease. 10.1016/j.leukres.2019.106284
NK/T Cell Lymphoma: Updates in Therapy. Suzuki Ritsuro Current hematologic malignancy reports PURPOSE OF REVIEW:Extranodal NK/T cell lymphoma (ENKL), nasal type, is a highly aggressive lymphoma which used to show a poor clinical outcome. Expression of P-glycoprotein on lymphoma cells of ENKL is a major reason for the refractoriness to conventional chemotherapy containing anthracycline. However, recent innovative approaches have improved the outcome and prognosis of ENKL. The purpose of this review is to summarize the proceedings of treatment. RECENT FINDINGS:Concurrent chemoradiotherapy containing platinum and several drugs including L-asparaginase, methotrexate, and alkylators shows excellent outcomes for the limited-stage ENKL. SMILE (steroid, methotrexate, ifosfamide, L-asparaginase, and etoposide) or other L-asparaginase-containing therapy is promising for advanced-stage ENKL, followed by either autologous or allogeneic hematopoietic stem cell transplantation. Anti-PD-1 or other immunological checkpoint inhibitors are recently reported to be effective for relapsed/refractory ENKL thought to be due to EBV-driven upregulation of PD-L1 expression. The prognosis of ENKL is therefore improving by the introduction of these strategies. The 5-year overall survival (OS) rate of limited stage was 63.2% [95% confidence interval (CI), 55.3 to 70.0%] before 2010, but was 79.4% (95% CI, 66.9 to 87.6%) in 2010 or after. However, there still exists a room for improvement, particularly for advanced-stage patients. The 2-year OS of advanced ENKL was 30.3% (95% CI, 19.5 to 41.7%) before 2010, but was 40.5% (95% CI, 24.8 to 55.8%) in 2010 or after. Optimal treatment scheme should further be explored. 10.1007/s11899-018-0430-5
Current treatment approaches for NK/T-cell lymphoma. Yamaguchi Motoko,Miyazaki Kana Journal of clinical and experimental hematopathology : JCEH Extranodal NK/T-cell lymphoma, nasal type (ENKL), is a form of lymphoma characterized by preferential extranodal involvement, Epstein-Barr virus (EBV) association, and geographic diversity in incidence. ENKL tumor cells express P-glycoprotein, which is related to multidrug resistance (MDR). This MDR phenomenon is thought to be the major reason why ENKL is resistant to anthracycline-containing chemotherapies and has led researchers to explore novel therapeutic strategies. Since the early 2000s, next-generation therapies, including upfront radiotherapy, chemotherapy, or concurrent chemoradiotherapy using non-MDR-related drugs, have markedly changed the management of ENKL. However, a recent large retrospective study in Japan revealed several limitations of next-generation therapies, in particular that they resulted in almost no improvement of early disease progression. This review will summarize the current management of ENKL, primarily based on clinical trial results, and provide clues for better future management. 10.3960/jslrt.17018
[Nasal NK/T-cell lymphoma: about two rare cases]. Bouayad Najoua,Oubelkacem Naoual,Bono Wafaa,Masbah Ouafae,Bouhafa Touria,Elmazghi Abderrahman,Khalid Hassouni The Pan African medical journal NK/T-cell lymphoma is a severe and rare disorder in Africa and in Europe. Treatment is based on radiotherapy and chemotherapy. We here report two cases of elderly patients aged 55 years and 52 years, respectively, with bilateral nasal obstruction associated with purulent rhinorrhea. Clinical examination showed cleft palate. Diagnosis was made on the basis of immunohistochemical examination of biopsies. Both patients underwent CHOEP chemotherapy followed by radiotherapy. Nasal NK/T-cell lymphoma is an aggressive type of non-Hodgkin's lymphoma with specific clinicopathologic features. Combining chemotherapy with radiotherapy in patients with advanced stage of the disease does not seem to improve survival compared with radiotherapy alone, which is the treatment of choice especially for localized stages. Nasal NK/T-cell lymphoma is rare. Diagnosis is based on immunohistochemical examination. Treatment includes chemotherapy and radiotherapy. This lymphoma has a poor overall prognosis, even with appropriate therapy. 10.11604/pamj.2018.30.141.7721