scholarly journals CD56− extranodal natural killer (NK)/T-cell lymphoma, nasal type presenting as skin ulcers in a white man

2016 ◽  
Vol 2 (5) ◽  
pp. 390-396 ◽  
Author(s):  
Charles Vainder ◽  
Jonhan Ho ◽  
Steven H. Swerdlow ◽  
Oleg E. Akilov
Oncotarget ◽  
2016 ◽  
Vol 7 (36) ◽  
pp. 58396-58404 ◽  
Author(s):  
Lei Zhang ◽  
Sucai Li ◽  
Sisi Jia ◽  
Feifei Nan ◽  
Zhaoming Li ◽  
...  

Blood ◽  
1997 ◽  
Vol 89 (12) ◽  
pp. 4501-4513 ◽  
Author(s):  
John K.C. Chan ◽  
V.C. Sin ◽  
K.F. Wong ◽  
C.S. Ng ◽  
William Y.W. Tsang ◽  
...  

Abstract Expression of the natural killer (NK) cell antigen CD56 is uncommon among lymphomas, and those that do are almost exclusively of non–B-cell lineage and show a predilection for the nasal and nasopharyngeal region. This study analyzes 49 cases of nonnasal CD56+ lymphomas, the largest series to date, to characterize the clinicopathologic spectrum of these rare neoplasms. All patients were Chinese. Four categories could be delineated. (1) Nasal-type NK/T cell lymphoma (n = 34) patients were adults 21 to 76 years of age (median, 50 years), including 25 men and 9 women. They presented with extranodal disease, usually in multiple sites. The commonest sites of involvement were skin, upper aerodigestive tract, testis, soft tissue, gastrointestinal tract, and spleen. Only 7 cases (21%) apparently had stage I disease. The neoplastic cells were often pleomorphic, with irregular nuclei and granular chromatin, and angiocentric growth was common. The characteristic immunophenotype was CD2+ CD3/Leu4− CD3ε+ CD56+, and 32 cases (94%) harbored Epstein-Barr virus (EBV). Follow-up information was available in 29 cases: 24 died at a median of 3.5 months; 3 were alive with relapse at 5 months to 2.5 years; and 2 were alive and well at 3 and 5 years, respectively. (2) Aggressive NK cell leukemia/lymphoma (n = 5) patients presented with hepatomegaly and blood/marrow involvement, sometimes accompanied by splenomegaly or lymphadenopathy. The neoplastic cells often had round nuclei and azurophilic granules in the pale cytoplasm. All cases exhibited an immunophenotype of CD2+ CD3/Leu4− CD56+ CD16− CD57− and all were EBV+. All of these patients died within 6 weeks. (3) In blastoid NK cell lymphoma (n = 2), the lymphoma cells resembled those of lymphoblastic or myeloid leukemia. One case studied for CD2 was negative and both cases were EBV−. One patient was alive with disease at 10 months and one was a recent case. (4) Other specific lymphoma types with CD56 expression (n = 8) included one case each of hepatosplenic γδ T-cell lymphoma and S100 protein+ T-cell lymphoproliferative disease and two cases each of T-chronic lymphocytic/prolymphocytic leukemia, lymphoblastic lymphoma, and true histiocytic lymphoma. All of these cases were EBV−. Six patients died at a median of 6.5 months. Nonnasal CD56+ lymphomas are heterogeneous, but all pursue a highly aggressive clinical course. The nasal-type NK/T-cell lymphoma and aggressive NK cell leukemia/lymphoma show distinctive clinicopathologic features and a very strong association with EBV. Blastoid NK cell lymphoma appears to be a different entity and shows no association with EBV.


Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 1704-1704
Author(s):  
Bing Xu ◽  
Rongwei Li ◽  
Pengcheng Shi ◽  
Huijuan Dong ◽  
Xutao Guo ◽  
...  

Abstract Background: Extranodal natural killer (NK)/T-cell lymphoma, nasal type (ENKTL) is quite rare in Western populations, but relatively common in East Asia (especially China) and Latin America. ENKTL is an aggressive disease with poor prognosis, requiring risk stratification. Similar symptoms, such as nasal obstruction and purulent nasal discharge, are found in patients with nasal NKTL and in patients with chronic rhinosinusitis. The images from contrast-enhanced computed tomography (CT) frequently show no prominent mass. As a result, the diagnosis is often delayed because it is frequently misdiagnosed as sinusitis. F-18 FDG PET/CT is a powerful imaging tool for diagnosis, staging, and evaluation of therapeutic effect in oncology. Aims: This study was designed to investigate the value of FDG PET/CT in the therapeutic management of extranodal natural killer (NK)/T-cell lymphoma, nasal type. Methods: A total of 26 patients with NK/T-cell lymphoma, nasal type were diagnosed according to morphologic and immunophenotypic criteria as specified in the World Health Organization (WHO) classification. All patients underwent FDG PET/CT and clinical information was obtained by review of medical records. Results: In 26 cases, all nasal/extranasal lesions were FDG-avid evidented to be neoplasm on CT scan and histopathological examinations. FDG-avid lesions in nasal/maxillary areas were uniformly more localized than demonstrated on CT scan, suggesting soft tissue masses on CT were partly due to inflammatory reaction. Among the 26 patients with definite diagnosis, 9 patients were re-staged on the basis of F18 FDG PET/CT with 5 patients down-staged and 4 patients up-staged. Statistical difference of the standardized uptake values (SUV) after 6 courses of chemotherapy and/or radiotherapy between the complete remission (CR) group and the partial remission (PR) group can be found (4.1±2.3 versus 7.8±1.7,P=0.006). The SUV value between pre-reatment and post-treatment were also of statistical significance in 22 patients (11.4±6.2 versus 5.6±2.1,P = 0.000).At a follow up of 18 months, patients got CR had a longer survival time than those got PR, stable disease (SD) or progress disease (PD) in 20 patients out of 26 patients.(median survival:310 days vs 284 days,(95% CI: 0.7156 to 1.365, p<0.05). Conclusions: Our preliminary study suggests that FDG PET/CT can provide more accurate information on the diagnosis, staged and therapeutic response assessment in extranodal natural killer (NK)/T-cell lymphoma, nasal type. FDG PET/CT can be an invaluable imaging modality in this clinical setting. Further investigation with large patients enrollment is warranted. Disclosures No relevant conflicts of interest to declare.


2018 ◽  
Vol 2018 ◽  
pp. 1-7
Author(s):  
Chih-Yi Liu ◽  
Hui-Chih Tsai

Natural killer (NK)/T-cell lymphoma is formally referred to as extranodal NK/T-cell lymphoma, nasal type (ENKTCL), in the 2008 and 2016 World Health Organization (WHO) classifications. NK/T-cell lymphoma, nasal type, is a rare but clinically important lymphoid neoplasm. It is the predominant type of extranodal lymphoma associated with the Epstein–Barr virus (EBV). NK/T-cell lymphoma is marked by a wide cytomorphological spectrum. The cytological findings may be so subtle that NK/T-cell lymphoma could possibly be easily overlooked. Here, we report a case of NK/T-cell lymphoma involving the sinonasal region with lymph node involvement. Fine needle aspiration of the neck lymph node and punch biopsy of the nasal mucosa were performed. The diagnosis of NK/T-cell lymphoma was confirmed based on pathological and immunohistochemical analyses, as well as in situ hybridization for EBV-encoded mRNA (EBER). The present case report underlines the importance of prompt clinicopathological assessment in suspected cases. The comparison of cytomorphologic features of NK/T-cell lymphoma in various specimens is presented.


2016 ◽  
Vol 17 ◽  
pp. 934-938 ◽  
Author(s):  
Ma\'in Ali Al Shawabkeh ◽  
Mansour Al Sulaiti ◽  
Hamad Al Sa\'ey ◽  
Shanmugam Ganesan

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