Extranodal natural killer/T-cell lymphoma with paraneoplastic eosinophilic myositis (original) (raw)
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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.
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 ...
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 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.
Frontiers in Oncology, 2022
Extranodal Natural Killer/T-cell lymphoma (ENKTL) is an extremely rare type of lymphoma which is highly lethal. It mainly affects the midline area unfolding as a necrotic granulomatous and extremely disfiguring lesion. There are two subtypes of (NKTL); the most common one is nasal which appears in the nasal cavity including the nasopharynx, oropharynx, parts of the aero digestive tract and Waldeyer’s ring. While the other rarer subtype, appears in sites like the skin, testis, gastrointestinal tract, salivary glands and muscle. ENKTL is popular for the expression of multidrug resistance-associated P-glycoprotein, which not only plays the main role at exporting many antitumor agents outside tumor cells, but also makes the disease hard to treat. It is commonly associated with Epstein-Barr virus (EBV) infection and commonly occurs in Asian populations. However, there is no single unified consensus yet as to what is the standardized treatment for ENKTL. Radiotherapy alone treatment, has ...
International Journal of Medicine and Public Health, 2015
Extranodal NK/T-cell Lymphoma of nasal type is a rare and comparatively a new entry among group of Non-Hodgkin lymphomas. The disease is characterized by a clinically aggressive course with involvement of upper aero-digestive tract and classical immune-phenotyping with CD2, CD3 and CD56 positivity. Being a rare entity, treatment entities are yet not formulated in guidelines. We hereby report a case of extranodal NK/T-cell lymphoma with predominant T cell markers who was initially treated with CHOP regime of non-Hodgkin lymphoma and later successfully treated with SMILE regime.
Cutaneous extranasal NK/T-cell lymphoma
Journal of the European Academy of Dermatology and Venereology, 2003
Malignant neoplasm from natural killer (NK) cells are characterized by their positivty for CD56 and absence of monoclonal TCR gene rearrangement. We present the case of a 54-year-old man with a fungous mass in his left flank whose histological examination was consistent with cutaneous extranasal T/NK cell lymphoma. We review the literature and also discuss the prognose and treatment of this variety of lymphoma.
An unusual case of lymphoma — a case of extranodal NK/T-cell lymphoma, nasal type
The Egyptian Journal of Otolaryngology
Background There is a great geographic variation of extranodal natural killer (NK)/T-cell lymphoma, nasal type (NNKTL) prevalence, with a much higher prevalence in the Asian and South American populations. According to our knowledge and searches, only one other case report/study of NNKTL has been published in South Africa. Case presentation We present a southern-African 31-year-old male residing in a township in the south of Johannesburg, South Africa. He presented with signs and symptoms similar to those of benign upper airway diseases. Further work-up of persisting signs and symptoms yielded a diagnosis of NNKTL. He was treated with the SMILE (steroids — dexamethasone, methotrexate, ifosfamide, L-asparaginase and etoposide) chemotherapy regimen, and sandwich radiotherapy was planned. He had a partial response to chemotherapy but unfortunately demised due to overwhelming sepsis prior to radiation therapy. Conclusion Making a diagnosis of NNKTL in resource-limited settings is challe...