NCCN Guidelines Insights: T-Cell Lymphomas, Version 2.2018 (original) (raw)
Related papers
Extranodal NK/T-cell lymphoma: diagnosis and treatment cues
Hematological Oncology, 2008
Extranodal NK/T-cell lymphoma, nasal type (ENKL) is mostly endemic to East Asia. It predominantly occurs in the nasal or paranasal areas and less frequently in the skin. Most of the tumours show NK-cell, but rarely T-cell, phenotypes. The Epstein-Barr virus (EBV) genome can be usually detected in lymphoma cells. Geographic localization of ENKL matches the endemic distribution of EBV, suggesting that EBV plays an important role in lymphomagenesis. Originally, NK-cell and T-cell types were believed to present the same clinicopathologic characteristics, but recent data suggest more aggressive characteristics for the NK-cell phenotype. Although ENKL is sensitive to radiotherapy, it shows a poorer response to chemotherapeutic agents than other lymphomas due to expression of p-glycoprotein. Therefore, new therapeutic approaches must be considered. Several new clinical trials are now being conducted in East Asia.
NHL - Extranodal T-cell lymphoma
National Journal of Maxillofacial Surgery, 2015
Lymphoma is the most common among blood cancers. The two main forms of lymphoma are Hodgkin lymphoma and non-Hodgkin lymphoma (NHL). Lymphoma occurs when cells of a type of lymphocyte (the cells of the immune system are called lymphocytes) travel to many parts of the body, including the lymph nodes, spleen, and bone marrow, and form a mass called a tumor. The body has two main types of lymphocytes that can develop into lymphomas: B lymphocytes (B cells) and T lymphocytes (T cells). [1] Lymphocytes account for 3-5% of all malignant tumors. NHLs account for 60% of all lymphomas; involvement of the nasal cavity and paranasal sinuses by these tumors is uncommon. [2] Extranodal NK/T-cell lymphomas, nasal type (ENKLs) are aggressive, locally destructive, midfacial necrotizing lesions characterized by extranodal involvement, particularly the nasal/paranasal area, and represent about 75% of all nasal lymphomas, the rest being B-cell lymphomas. [3,4] The lesion typically causes local destruction of cartilage, bone, and soft tissues. [5] Incidence of ENKL varies considerably in different parts of the world, but it remains a rare disease since its first description in 1933. However, the number of new, diagnosed cases per year is on the increase, due to improved knowledge of this disease. [6] ENKL is a rare type of NHL. This type of lymphoma was previously called angiocentric lymphoma. It is more common in Asian and Central and South American countries than it is in North America. ENKL can develop in either T cells or natural killer (NK) cells, which attack foreign cells. Sometimes it is difficult to tell which cells-T cells or NK cells-are present. [7] ENKLs are subcategorized into nasal and nasal-type NK/T-cell lymphomas according to the major site of anatomic involvement. In limited cases, NK/T-cell lymphomas may predominantly occur in extranasal sites without involvement of the nasal cavity or nasopharynx. [6] Advances in tumor cell biology have led to the ability to subclassify NHLs using the World Health Organization (WHO) classification of lymphoma. [6] The terminology becomes more precise as our ability to genetically characterize these tumors improves. [8]
Extranodal Natural Killer/T-Cell Lymphomas: Current Approaches and Future Directions
Journal of Clinical Medicine
Extranodal natural killer/T(NK/T)-cell lymphoma (ENKTL) is a rare subtype of non-Hodgkin lymphoma that typically presents with an isolated nasal mass, but a sizeable minority present with advanced stage disease and have a significantly poorer prognosis. Those with limited disease are standardly treated with chemotherapy and radiation while those with advanced stage disease are treated with L-asparaginase containing chemotherapy regimens. The addition of modern radiation therapy techniques and the incorporation of L-asparaginase into chemotherapy regimens have significantly improved outcomes in this disease, but relapses and death from relapsed disease remain frequent. Given the high rate of relapse, several novel therapies have been evaluated for the treatment of this disease. In this review, we explore the current standard of care for ENKTL as well as novel therapies that have been evaluated for its treatment and the biologic understanding behind these therapies.
The American Journal of Surgical Pathology, 2012
Extranodal NK/T-cell lymphoma (ENKTL), nasal type, may be of NK or T-cell origin; however, the proportion of T-ENKTLs and whether they are of ab or gd type remains uncertain. To elucidate the cell of origin and detailed phenotype of ENKTL and assess any clinicopathologic associations, 67 cases of ENKTL from Thailand were investigated, together with 5 gd enteropathy-associated T-cell lymphomas (EATLs) for comparison. In all, 70% of the ENKTL were T-cell receptor (TCR) b,g and, in cases tested, d negative (presumptive NK origin); 5% were TCR gd + , 3% were TCR ab + , 1% were TCR ab/gd + , and 21% were indeterminate. Out of 17 presumptive NK-ENKTLs tested, 3 had clonal TCR rearrangements. All cases were EBV + and TIA-1 + ; >85% were positive for CD3, CD2, granzyme B, pSTAT3, and Lsk/MATK; and all were CD16 À . Presumptive NK-ENKTLs had significantly more frequent CD56 (83% vs. 33%) and CXCL13 (59% vs. 0%) but less frequent PD-1 (0% vs. 40%) compared with T-ENKTLs. Of the NK-ENKTLs, 38% were Oct-2 + compared with 0% of T-ENKTLs, and 54% were IRF4/MUM1 + compared with 20% of T-ENKTLs. Only ab T-ENKTLs were CD5 + . Intestinal ENKTLs were EBV + and had significantly more frequent CD30, pSTAT3, and IRF4/MUM1 expression but less frequent CD16 compared with gd EATL. Significant adverse prognostic indicators included a primary non-upper aerodigestive tract site, high stage, bone marrow involvement, International Prognostic Index Z2, lack of radiotherapy, Ki67 >40%, and CD25 expression. The upper aerodigestive tract ENKTLs of T-cell origin compared with those of presumptive NK origin showed a trend for better survival. Thus, at least 11% of evaluable ENKTLs are of T-cell origin. Although T-ENKTLs have phenotypic and some possible clinical differences, they share many similarities with ENKTLs that lack TCR expression and are distinct from intestinal gd EATL.
The Lancet Haematology, 2020
Background Extranodal natural killer (NK) T-cell lymphoma (ENKTL) is a unique clinicopathological entity, typically associated with poor survival outcomes. Most published data have come from east Asian study groups, with little information available from international cohorts. The effects of treatment advances on routine clinical practice across continental territories has not been clear. We aimed to improve understanding of the clinical characteristics and outcomes of patients with ENKTL. Methods We did a substudy of patients with ENKTL from the T-cell Project, a global prospective cohort study. The T-cell Project registered consecutively diagnosed adults (>18 years) with newly diagnosed, untreated mature T-cell or NK lymphomas (WHO 2001 or 2008 classifications) from 74 centres in 13 countries (in Asia, Europe, North America, and South America). In total, 1695 patients with mature T-cell or NK lymphomas were enrolled between Oct 12, 2006 and Feb 28, 2018 in the T-cell Project. The first patient with ENKTL was enrolled on Feb 15, 2007, and the last on May 26, 2017. Data on baseline characteristics, first-line treatment, treatment response, and survival outcomes were recorded in a central database (locked March 30, 2019). The primary outcome was 5-year overall survival. The T-cell Project is registered on ClinicalTrials.gov, NCT01142674. Findings 166 patients were diagnosed with ENKTL, comprising 11% of 1553 eligible registered cases and distributed across 40 participating centres in four continents. At a median follow-up of 44 months (IQR 20-61), overall survival at 5 years was 54% (95% CI 44-63) in patients with nasal disease (n=98) and 34% (27-46) in patients with extranasal disease (n=68). Interpretation To our knowledge, this study presents the largest international cohort of patients with ENKTL. We describe a clinically significant improvement in the survival of patients with ENKTL treated in routine clinical practice over the past decade, likely to be attributable to the increasing use of treatment protocols specific for ENKTL.
Journal of Oral and Maxillofacial Pathology, 2011
These lymphomas are uncommon neoplasms in the United States, representing approximately 1.5% of all lymphomas. A higher incidence, however, has been reported in Asian and South American countries, especially Peru. In these areas, primary NHL accounts for approximately 6.7-8.0% of all lymphomas. [5] ENKL is typically observed in adults but may be seen in children. Studies have shown a male to female ratio of 2:1 to 3:1. Tumors are most common in the nasal cavity but other sites may include the skin, GIT, testis, kidney, upper respiratory tract and rarely the eye/orbit. Involvement of the regional lymph nodes is unusual until the tumor disseminates. [3]
Journal of clinical oncology : official journal of the American Society of Clinical Oncology, 2017
Purpose To elucidate the management and outcomes of patients with extranodal natural killer/T-cell lymphoma, nasal type (ENKL), who were diagnosed between 2000 and 2013 in Japan. Patients and Methods Data from 358 patients with ENKL diagnosed between 2000 and 2013 from 31 institutes were retrospectively analyzed. Results Patients' median age was 58 years, and 257 (72%) had localized disease. The most common first-line treatment was radiotherapy with dexamethasone, etoposide, ifosfamide, and carboplatin (RT-DeVIC) (66%) for localized ENKL and L-asparaginase-containing chemotherapy (30%) for advanced ENKL. With a median follow-up of 5.8 years, overall survival (OS) rates at 5 years for localized and advanced ENKL were 68% and 24%, respectively. The prognostic index of natural killer lymphoma was validated in our study, although only 4% of patients with localized ENKL were classified as high risk. With a median follow-up of 5.6 years, OS and progression-free survival at 5 years in ...