|Year : 2015 | Volume
| Issue : 3 | Page : 665
Efficacy of pegaspargase in extra nodal natural killer/T-cell lymphoma nasal type: A case report from China
Xingan Xiong1, Zhiqiang Cai2, Jiyuan Yang2, Xiaoyan Shu2
1 Department of Pharmacology, The First Affiliated Hospital, Yangtze University, Jingzhou, Hubei, China
2 Department of Oncology, The First Affiliated Hospital, Yangtze University, Jingzhou, Hubei, China
|Date of Web Publication||9-Oct-2015|
Department of Oncology, The First Affiliated Hospital, Yangtze University, Jingzhou, Hubei
Source of Support: None, Conflict of Interest: None
Extranodal natural killer (NK)/T-cell lymphoma, nasal type, is a rare and highly aggressive disease with a grim prognosis. There is no known satisfactory treatment. The author herein to report one case of L-asparaginase extranodal NK/T-cell lymphoma primary treated with L-asparaginase methotrexate and dexamethasone.
Keywords: Initial chemotherapy, L-asparaginase, natural killer/T-cell lymphoma
|How to cite this article:|
Xiong X, Cai Z, Yang J, Shu X. Efficacy of pegaspargase in extra nodal natural killer/T-cell lymphoma nasal type: A case report from China. J Can Res Ther 2015;11:665
|How to cite this URL:|
Xiong X, Cai Z, Yang J, Shu X. Efficacy of pegaspargase in extra nodal natural killer/T-cell lymphoma nasal type: A case report from China. J Can Res Ther [serial online] 2015 [cited 2020 May 28];11:665. Available from: http://www.cancerjournal.net/text.asp?2015/11/3/665/140750
| > Introduction|| |
Extranodal natural killer (NK)/T-cell lymphoma, nasal type, is a rare and severe malignancy. It is an extranodal lymphoma usually with an immature NK-cell phenotype and Epstein-Barr virus positivity. It is classically characterized by a cytoplasmic CD3 ε phenotype, with no surface CD3 or T-cell receptor expression, no T-cell receptor gene rearrangements, an activated cytotoxic profile with perforin, granzyme B, and T-cell intracellular antigen-1 expression, and frequent CD56 expression. ,,
There is a geographic predominance in Asia, where it comprises 3-8% of nonHodgkin's lymphoma, and in Central and South America.  More common in males, it may affect children, or adults, with a median age of diagnosis the fifth decade.  There are two clinical entities, nasal and extranasal NK/T cell lymphoma, which diverge in clinical presentation, treatment, and prognosis.  Because disease incidence is rare even in prevalent areas, there has been no randomized controlled trial, and most treatment protocols are consensus-guided.  Localized NK/T-cell lymphomas often respond to radiotherapy , or to concurrent radiation and chemotherapy,  but relapse is common. Chemotherapy protocols used for lymphomas of other histologic subtypes are poorly effective, at least in part, because of frequent multidrug resistance gene expression by tumor cells. 
We report one case of initial treated with L-asparaginase-containing regimen for patients with disseminated NK/T lymphoma. We combine L-asparaginase with methotrexate, a drug insensitive to the multidrug resistance pathway, because of its well-known synergistic effect with asparaginase in acute lymphoblastic leukemia and its ability to prevent central nervous system involvement. Dexamethasone was added because T-cell lymphomas are usually sensitive to corticosteroids and dexamethasone seems to be associated with a lower risk of thrombosis when given with L-asparaginase. 
| > Case report|| |
A 73-year-old Chinese man noted fullness in the left nasal cavity and difficulty breathing on that side. Computed tomography (CT) scan revealed a soft tissue opacity filling the left maxillary frontal and ethmoid sinuses [Figure 1]. Excisional biopsy showed extranodal NK/T-cell lymphoma. Immunophenotyping revealed CD3 + , CD45RO + , CD79α− , CD56 + , GranzymeB + , CD30− , CyclinD1− , Ki67 + :50-60%, EBER (By fluorescence in situ hybridization)- [Figure 2]. He denied fevers, chills or night sweats, but had 7-8 pound weight loss over 2 months. His physical exam was remarkable only presented with left neck mass. Excisional biopsy showed extranodal NK/T-CELL lymphoma. Bone marrow biopsy and CT scan showed no other evidence of lymphoma. He was diagnosed with stage II E extranodal NK/T lymphoma, nasal type. He received two 21-day cycles AspaMetDex (pegaspargase with methotrexate and dexamethasone) regime. Pegaspargase was initiated at 2500 units/m 2 of body surface area on days 1, 14, intramuscularly, plus methotrexate 2 g/m 2 on day 1, and oral dexamethasone 20 mg from day 1 to 4. After two cycles, the patient can breath well, responses assessed by CT scan is a partial response,  there are a little soft tissue in the left nasal cavity according the CT scan [Figure 3]. Physical examination showed that the mass in the left neck also almost disappeared. Unfortunately, the treatment discontinued because of health expense.
|Figure 2: Immunohistochemistry (IHC) and fluorescence in situ hybridization (FISH) of tumor: CD3+, CD45RO+, CD79α−, CD56+, GranzymeB+, CD30−, CyclinD1−, Ki67+:50-60%, EBER (By FISH)−(IHC ×40) CD20−|
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| > Discussion|| |
In a recent international multicenter series reported by the International Peripheral T-cell Lymphoma Project, the median survival time was only 7.8 months.  There is no consensus on the optimal therapeutic regimen in the salvage setting. Etoposide, ifosfamide, and methotrexate based regimens exhibited a complete response rate of ~40% with median disease free survival of 12 months in one study,  and a 44% response rate in 32 patients with median overall survival of 8.2 months and time to treatment failure of 3.7 months in another.  Chinese group who first reported the use of asparaginase to treat NK/T-cell lymphoma, in four retrospective series. ,,, However, in these studies, the patients had localized disease and received irradiation after asparaginase, making it impossible to determine whether asparaginase or irradiation was responsible for the excellent response rate. Jaccard et al.  had reported that L-asparaginase with methotrexate and dexamethasone in patients with refractory or relapsing extranodal NK/T-cell lymphoma is more effective. Reyes et al.  show that L-asparaginase is a viable treatment alternative in relapsed NK/T-extranodal lymphoma.
| > Conclusion|| |
There are many research has proved the efficacy in relapsed NK/T-extranodal lymphoma. In our case, we first report the L-asparaginase is also effective in the initial treatment of extranodal NK/T-cell lymphoma nasal type. Because of the limited cases, so first-line L-asparaginase combination therapy for extranodal NK/T-cell lymphoma initial treatment need further study.
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[Figure 1], [Figure 2], [Figure 3]