Diagnostic approach to extranodal NK/T-cell lymphomas using a rare case of non-nasal type extranodal NK/T-cell lymphoma with gamma/delta-T-cell phenotype

2018 ◽  
Vol 101 ◽  
pp. S23
Author(s):  
Cristina Lamos ◽  
Edgar Dippel
2019 ◽  
Vol 11 (1) ◽  
Author(s):  
Ana Caballero ◽  
Silvana Novelli ◽  
Anna Mozos ◽  
Pilar Garcia Muret ◽  
Anna Monter ◽  
...  

Within T-cell lymphomas (TCL) there are 2 entities expressing gamma-delta TCR: hepatosplenic gamma-delta T-cell lymphoma (HSGDTL) and the primary cutaneous gamma-delta T-cell lymphoma (PCGDTL). PCGDTL is a rare form of Tcell lymphoma with specific tropism for skin that have a dismal prognosis. Although even rarer, there have been reports of TCL with loss of expression of the TCR, which have been termed peripheral TCL TCR-silent type. We report the case of a cutaneous TCR-silent type lymphoma associated to a clonal plasma cell proliferation with an ominous outcome that led to a lot of discussion in its classification. Due to the aggressiveness of the disease and the scant evidence about therapy in this strange entity the outcome was fatal. We report a unique case of a TCR-silent cutaneous TCL with an exceptional histopathology, prolonged clinical evolution and a subsequent plasma cell clonal expansion.


2011 ◽  
Vol 44 (9) ◽  
pp. 1205-1211
Author(s):  
Takehiro Fujii ◽  
Hiroyuki Kato ◽  
Makoto Suzaki ◽  
Takashi Noguchi ◽  
Hiroshi Imai

2019 ◽  
Author(s):  
Joohwan Park ◽  
Miso Kim ◽  
Tae-Min Kim ◽  
Bhumsuk Keam ◽  
Dong-Wan Kim ◽  
...  

Abstract Systemic inflammatory response index such as neutrophil-to-lymphocyte ratio (NLR) or platelet-to-lymphocyte ratio (PLR) has been investigated as prognostic indicators in various tumors including diffuse large B-cell and peripheral T-cell lymphomas. The aim of this study is to evaluate the prognostic values of the pretreatment NLR and PLR in patients with advanced, extranodal NK/T-cell lymphoma, nasal type (NTCL). Sixty patients with newly diagnosed, stage III/IV NTCL between January 2000 and August 2015 were evaluated retrospectively. Correlations of pre-NLR and –PLR with other clinical factors were investigated. Kaplan-Meier plot was used to analyze survivals according to pre-NLR and -PLR levels. The prognostic effects of variables were investigated using a Cox proportional regression model. Based on the optimized cut-off levels, patients were classified into two different groups. After a median follow up of 15 months, the median overall survival (OS) in the low and high pre-NLR groups was 33.4 and 6.6 months, respectively. In multivariate analysis, high pre-NLR was significantly associated with reduced OS (hazard ratio [HR] 3.59, 95% CI 1.77-7.26; p < .001). On the contrary, the median OS in the low and high pre-PLR was 6.2 and 22.3 months, respectively. Multivariate analysis revealed that low pre-PLR was in a significant correlation with the worse OS (HR 3.78, 95% CI 1.79-7.95; p < .001). The high pre-NLR and low pre-PLR are independent poor prognostic factors for reduced survivals of patients with advanced NTCL, and might provide additional prognostic powers to identify high-risk, advanced NTCL patients.


2002 ◽  
Vol 126 (12) ◽  
pp. 1527-1529
Author(s):  
Kameel F. Totonchi ◽  
George Engel ◽  
Elliot Weisenberg ◽  
Douglas P. Rhone ◽  
William R. Macon

Abstract The majority of primary testicular lymphomas are of B-cell type. Other primary lymphomas are rarely encountered in the testes. Natural killer (NK)/T-cell lymphomas of nasal type are aggressive extranodal lymphomas associated with Epstein-Barr virus infection that are usually encountered in the upper aerodigestive tract. They also occur in the skin, soft tissue, and colon. Primary testicular NK/T-cell lymphomas are rarely reported. We describe the case of a 66-year-old Korean man who presented with right-sided painless testicular enlargement and underwent radical orchiectomy. Histologic examination revealed an angiocentric and angioinvasive infiltrate of medium to large tumor cells with moderately abundant pale pink cytoplasm and folded and indented pleomorphic nuclei. Paraffin immunohistochemical studies showed positivity of the tumor cells for CD45, TIA-1, granzyme B, CD56, and CD3ɛ. In situ hybridization showed diffuse positivity for Epstein-Barr virus–encoding RNA. The results of gene rearrangement studies for the γ chain of the T-cell receptor were negative. The results of paraffin immunohistochemical studies for CD20, CD8, CD45RO, beta f1, and ALK-1 were negative. An extensive workup showed no evidence of lymphoma outside the testes. We report a rare case of primary testicular NK/T-cell lymphoma of the nasal type of true NK-cell origin.


2016 ◽  
Vol 7 (8) ◽  
pp. 364-367 ◽  
Author(s):  
Rahul Anil ◽  
Sana Grover ◽  
Adarsh Hiremath

2008 ◽  
Vol 11 (1) ◽  
pp. 50-54 ◽  
Author(s):  
Bill H. Chang ◽  
Linda Stork ◽  
Guang Fan

Mature T-cell neoplasms are unusual in the pediatric population. The majority of these neoplasms in the United States are anaplastic large cell lymphomas (ALCL) characterized by CD30 and anaplastic lymphoma kinase-1 expression. Extranodal natural killer/T (NK/T)-cell lymphomas, nasal type, are extremely rare. Extranodal NK/T-cell lymphomas often express CD56, are associated with Epstein-Barr virus, and are negative for CD30. Clinically, extranodal NK/T-cell lymphomas are much more aggressive than ALCL, and require different treatment strategies. The authors present an adolescent male with a CD56 negative extranodal NK/T-cell lymphoma, nasal type. The lymphoma was partially positive for CD30, diffusely positive for EBV by in situ hybridization, and clonal for T-cell receptor gene rearrangement and cytogenetic abnormalities. The patient was aggressively treated with chemotherapy, surgery, and radiation. More than 2 years from completion of the therapy, the patient remains disease free. This case highlights the importance and difficulty of accurate identification of this type of rare tumor. We further present the literature review and discuss the diagnostic criteria for extranodal NK/T lymphoma using morphologic, immunologic, molecular, and cytogenetic information.


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