Improved prognosis of extranodal NK/T cell lymphoma, nasal type of nasal origin but not extranasal origin

Yamaguchi, Motoko; Suzuki, Ritsuro; Miyazaki, Kana; Amaki, Jun; Takizawa, Jun; Sekiguchi, Nodoka; Kinoshita, Shiori; Tomita, Naoto; Wada, Hideho; Kobayashi, Yukio; Niitsu, Nozomi; Ando, Toshihiko; Maeda, Takeshi; Saito, Bungo; Matsuoka, Hiroshi; Sakai, Rika; Kubota, Nobuko; Yasukawa, Masaki; Kameoka, Yoshihiro; Asano, Naoki; Oguchi, Masahiko; Katayama, Naoyuki

doi:10.1007/s00277-019-03689-9

Cited by 22 publications

(31 citation statements)

References 23 publications

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“…It is typically extranodal and divided by primary site as nasal or extranasal [4]. Despite advances in therapy, extranasal NKTCL has a poor prognosis [5]. Clonal episomal EBV infection is integral to NK-cell lymphomagenesis, and its plasma DNA quantification has been used to assess burden of disease and response to treatment [6,7].…”

Section: Discussionmentioning

confidence: 99%

Combined immune checkpoint blockade and radiotherapy induces durable remission in relapsed natural killer/T-cell lymphoma: a case report and review of the literature

et al. 2021

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Background Extranodal natural killer/T-cell lymphoma is a rare, aggressive non-Hodgkin lymphoma that is treated upfront mostly with L-asparaginase containing regimens. Relapsed extranodal natural killer/T-cell lymphoma is associated with a poor prognosis, and there is no established standard of care. Case presentation We report the case of a 72 year-old white male with a distant extranasal relapse of extranodal natural killer/T-cell lymphoma that has been managed successfully with a combination of radiation and immune checkpoint blockade with pembrolizumab. Pseudoprogression with new skin and bone lesions on positron emission tomography imaging was encountered during this Caucasian patient’s immunotherapy and was successfully managed with supportive care and continuation of immune checkpoint blockade. Conclusions The patient has been in complete clinical, radiologic, and molecular remission for close to 3 years and has not had any immune-related adverse effects. Pseudoprogression is a clinical challenge that can be encountered while patients are treated with immunotherapy, and astute clinical acumen is needed for accurate management. We believe this is the longest duration of response to immune checkpoint blockade in relapsed extranodal natural killer/T-cell lymphoma reported to date in literature. There is a strong biologic rationale in combining radiation with immunotherapy. The optimal timing, dose, and duration of radiation combined with immunotherapy in extranodal natural killer/T-cell lymphoma need to be prospectively evaluated.

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Section: Discussionmentioning

confidence: 99%

Combined immune checkpoint blockade and radiotherapy induces durable remission in relapsed natural killer/T-cell lymphoma: a case report and review of the literature

et al. 2021

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“…In general, factors correlating to a poor prognosis in oropharyngeal lymphomas include: old age, advanced stage, B-symptoms, tumors originating from the soft palate and T-cell lymphomas (18). Especially extranodal NKTCL located outside the nasal cavity shows poor prognosis with reports of a 2-year overall survival (OS) of 34% and a 5-year OS of only 12% (21).…”

Section: Discussionmentioning

confidence: 99%

Lymphoma of the Uvula: Clinical, Morphological, Histopathological, and Genetic Characterization. A Nationwide Danish Study From 1980 to 2019

et al. 2021

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Background: In the head and neck region the uvula is a rare site for extranodal lymphomas to develop. In this national study, we present six cases and provide an overview of the current literature, characterizing the clinical and histopathological features of lymphomas involving this location.Materials and Methods: Clinical information was obtained retrospectively from patient records in a nationwide Danish study covering from 1980 through 2019. In order to validate the diagnoses, uvular tissue specimens were examined histologically and immunohistochemically and if relevant for subtyping, cytogenetic rearrangements were investigated.Results: We present six cases of lymphomas involving the uvula, of which four of the cases were diagnosed with a B-cell lymphoma (two diffuse large B-cell lymphomas, one extranodal marginal zone B-cell lymphoma and one Mantle cell lymphoma), while two were diagnosed with a T-cell lymphoma (one peripheral T-cell lymphoma and one natural killer/T-cell lymphoma). Presenting symptoms included swelling, pain and ulceration of the uvula. Treatment was comprised of radiotherapy and/or chemotherapy, with T-cell lymphomas showing a poorer outcome than B-cell lymphomas.Conclusion: Lymphoma of the uvula is rare, with few case reports being reported in the literature. The most frequent histological subtypes reported are extranodal marginal zone B-cell lymphoma and peripheral T-cell lymphoma. When encountering a swollen, painful and/or ulcerated uvula, the clinician should always consider malignancy as a possible cause. Lymphoma of the uvula is a possible diagnosis and if this is the case, there is a high risk of disseminated disease at the time of diagnosis.

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“…Relatively, patients with advanced disease had a dismal outcome, with a median survival of only several months even asparaginase-based chemotherapy was used (3,21). Besides, asparaginase-based chemotherapy did not seem to improve the survival of patients with extranasal NKTCL (24,25). Thus, novel treatment strategies are urgently needed for NKTCL patients with advanced disease, especially asparaginaseresistant disease.…”

Section: Discussion and Literature Reviewmentioning

confidence: 99%

Durable Response to Sintilimab and Chidamide in a Patient With Pegaspargase- and Immunotherapy-Resistant NK/T-Cell Lymphoma: Case Report and Literature Review

et al. 2020

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The prognosis of patients with relapsed/refractory NK/T-cell lymphoma (NKTCL) is dismal. Immunotherapy has showed encouraging anti-tumor activity in patients with asparaginase-resistant NKTCL; however, only a portion of patients benefit and the median response duration is rather short. Treatment strategies have not been identified for immunotherapy-resistant NKTCL. We describe a patient with primary cutaneous NKTCL experienced disease progression after pegaspargase-based chemotherapy and PD-1 inhibitor (sintilimab)-based immunotherapy. Following a combined treatment of sintilimab and the HDAC inhibitor chidamide, the patient achieved a durable complete molecular response with mild toxicity. This case indicates that the combination of PD-1 inhibitor and HDAC inhibitor might be a treatment choice for immunotherapy-resistant NKTCL.

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Improved prognosis of extranodal NK/T cell lymphoma, nasal type of nasal origin but not extranasal origin

Cited by 22 publications

References 23 publications

Combined immune checkpoint blockade and radiotherapy induces durable remission in relapsed natural killer/T-cell lymphoma: a case report and review of the literature

Combined immune checkpoint blockade and radiotherapy induces durable remission in relapsed natural killer/T-cell lymphoma: a case report and review of the literature

Lymphoma of the Uvula: Clinical, Morphological, Histopathological, and Genetic Characterization. A Nationwide Danish Study From 1980 to 2019

Durable Response to Sintilimab and Chidamide in a Patient With Pegaspargase- and Immunotherapy-Resistant NK/T-Cell Lymphoma: Case Report and Literature Review

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