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Extranodal NK-T Cell Lymphoma, Nasal Type: Retrospective Analysis of Real-World Data

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Indian Journal of Hematology and Blood Transfusion Aims and scope Submit manuscript

Abstract

Peripheral T cell lymphomas constitute nearly 15% of all cases on non-Hodgkin lymphoma. Of these, NK-T cell lymphoma nasal type is a rare and aggressive form. We present our experience of 16 patients of NK/T cell lymphoma which constituted approximately 1% of all lymphoma (N = 1590) cases treated at our center. Male to female ratio was 4.3:1. Median age of presentation was 42 years. Early Stage patients (n = 11) were treated with DeVIC regimen (n = 10) and SMILE (n = 1) chemotherapy and RT to all the patients. Advanced stage patients were treated with SMILE regimen (n = 4) and ICE and local RT (n = 1) with one treatment related mortality. The presence of B-symptoms adversely affected survival. The estimated median PFS and OS were 39 and 49 months respectively. Overall survival was not reached in Limited Stage patients (stage 1 and 2) and 8 months in patients with advanced stage (stage IV) (p = 0.001). According to the new CSWOG staging (retrospectively applied), comparing the Limited versus Extensive Stage, the earlier group has a significantly better estimated PFS (p = 0.020) and OS (p = 0.007). ENKTL is a rare malignancy with aggressive course. B-symptoms portend a poor prognosis to patients with this aggressive lymphoma. The new staging system helps estimate survival better.

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References

  1. Chim CS, Ooi GC, Shek TW, Liang R, Kwong YL (1999) Lethal midline granuloma revisited: nasal T/Natural-killer cell lymphoma. J Clin Oncol Off J Am Soc Clin Oncol 17(4):1322–1325

    Article  CAS  Google Scholar 

  2. Campo E, Swerdlow SH, Harris NL, Pileri S, Stein H, Jaffe ES (2011) The 2008 WHO classification of lymphoid neoplasms and beyond: evolving concepts and practical applications. Blood 117(19):5019–5032

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  3. Kwong Y-L, Khong P-L (2011) Central palatal perforation in nasal natural killer cell lymphoma. Br J Haematol 152(1):2

    Article  PubMed  Google Scholar 

  4. Takata K, Hong M-E, Sitthinamsuwan P, Loong F, Tan S-Y, Liau J-Y et al (2015) Primary cutaneous NK/T-cell lymphoma, nasal type and CD56-positive peripheral T-cell lymphoma: a cellular lineage and clinicopathologic study of 60 patients from Asia. Am J Surg Pathol 39(1):1–12

    Article  PubMed  Google Scholar 

  5. Chim C-S, Ma ESK, Loong F, Kwong Y-L (2005) Diagnostic cues for natural killer cell lymphoma: primary nodal presentation and the role of in situ hybridisation for Epstein–Barr virus encoded early small RNA in detecting occult bone marrow involvement. J Clin Pathol 58(4):443–445

    Article  PubMed  PubMed Central  Google Scholar 

  6. Au WY, Chan AC, Kwong YL (1998) Scrotal skin ulcer in a patient with a previous tonsillectomy because of natural killer cell lymphoma. Am J Dermatopathol 20(6):582–585

    Article  CAS  PubMed  Google Scholar 

  7. Kwong Y-L (2005) Natural killer-cell malignancies: diagnosis and treatment. Leukemia 19(12):2186–2194

    Article  CAS  PubMed  Google Scholar 

  8. Kimura H, Ito Y, Kawabe S, Gotoh K, Takahashi Y, Kojima S et al (2012) EBV-associated T/NK-cell lymphoproliferative diseases in nonimmunocompromised hosts: prospective analysis of 108 cases. Blood 119(3):673–686

    Article  CAS  PubMed  Google Scholar 

  9. Yamaguchi M, Kita K, Miwa H, Nishii K, Oka K, Ohno T et al (1995) Frequent expression of P-glycoprotein/MDR1 by nasal T-cell lymphoma cells. Cancer 76(11):2351–2356

    Article  CAS  PubMed  Google Scholar 

  10. Lin T, Hong H, Liang C, Huang H, Guo C, Tian Y et al (2014) Extranodal natural killer T-cell lymphoma, nasal-type—a new staging system from CSWOG—a multicenter study. J Clin Oncol 32(15_suppl):8552

    Article  Google Scholar 

  11. Wood PB, Parikh SR, Krause JR (2011) Extranodal NK/T-cell lymphoma, nasal type. Proc Bayl Univ Med Cent 24(3):251–254

    Article  PubMed  PubMed Central  Google Scholar 

  12. Au W, Weisenburger DD, Intragumtornchai T, Nakamura S, Kim W-S, Sng I et al (2009) Clinical differences between nasal and extranasal natural killer/T-cell lymphoma: a study of 136 cases from the International Peripheral T-Cell Lymphoma Project. Blood 113(17):3931–3937

    Article  CAS  PubMed  Google Scholar 

  13. Chan JK, Sin VC, Wong KF, Ng CS, Tsang WY, Chan CH et al (1997) Nonnasal lymphoma expressing the natural killer cell marker CD56: a clinicopathologic study of 49 cases of an uncommon aggressive neoplasm. Blood 89(12):4501–4513

    Article  CAS  PubMed  Google Scholar 

  14. Tse E, Kwong Y-L (2015) Nasal NK/T-cell lymphoma: RT, CT, or both. Blood 126(12):1400–1401

    Article  CAS  PubMed  Google Scholar 

  15. Yang Y, Zhu Y, Cao J-Z, Zhang Y-J, Xu L-M, Yuan Z-Y et al (2015) Risk-adapted therapy for early-stage extranodal nasal-type NK/T-cell lymphoma: analysis from a multicenter study. Blood 126(12):1424–1432 (quiz 1517)

    Article  CAS  PubMed  Google Scholar 

  16. Ueda K, Nishimura N, Mishima Y, Nitta H, Kusano Y, Yokoyama M et al (2014) RT-DeVIC therapy is effective for localized NK/T cell lymphoma patiens. Blood 124(21):1702

    Article  Google Scholar 

  17. Hatayama Y, Aoki M, Kawaguchi H, Narita Y, Hirose K, Sato M et al (2013) Impact of dexamethasone, etoposide, ifosfamide and carboplatin as concurrent chemoradiotherapy agents for nasal natural killer/T-cell lymphoma. Mol Clin Oncol 1(4):680–684

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  18. Ando M, Sugimoto K, Kitoh T, Sasaki M, Mukai K, Ando J et al (2005) Selective apoptosis of natural killer-cell tumours by l-asparaginase. Br J Haematol 130(6):860–868

    Article  CAS  PubMed  Google Scholar 

  19. Yamaguchi M, Kwong Y-L, Kim WS, Maeda Y, Hashimoto C, Suh C et al (2011) Phase II study of SMILE chemotherapy for newly diagnosed stage IV, relapsed, or refractory extranodal natural killer (NK)/T-cell lymphoma, nasal type: the NK-Cell Tumor Study Group study. J Clin Oncol Off J Am Soc Clin Oncol 29(33):4410–4416

    Article  CAS  Google Scholar 

  20. Kwong Y-L, Kim WS, Lim ST, Kim SJ, Tang T, Tse E et al (2012) SMILE for natural killer/T-cell lymphoma: analysis of safety and efficacy from the Asia Lymphoma Study Group. Blood 120(15):2973–2980

    Article  CAS  PubMed  Google Scholar 

  21. Li X, Cui Y, Sun Z, Zhang L, Li L, Wang X et al (2016) DDGP versus SMILE in newly diagnosed advanced natural killer/T-cell lymphoma: a randomized controlled, multicenter, open-label study in China. Clin Cancer Res Off J Am Assoc Cancer Res 22(21):5223–5228

    Article  CAS  Google Scholar 

  22. Kim M, Kim TM, Kim KH, Keam B, Lee S-H, Kim D-W et al (2015) Ifosfamide, methotrexate, etoposide, and prednisolone (IMEP) plus L-asparaginase as a first-line therapy improves outcomes in stage III/IV NK/T cell-lymphoma, nasal type (NTCL). Ann Hematol 94(3):437–444

    Article  CAS  PubMed  Google Scholar 

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Acknowledgements

We would like to acknowledge an entire team of hemato-oncology and bone marrow transplant unit, Rajiv Gandhi Cancer Institute and Research Centre for their continuous support for retrieving records of eligible patients.

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Correspondence to Narendra Agrawal.

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All procedures performed in study involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. The study was approved by our Institutional Review Board (IRB).

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This is a retrospective study hence for this type of study formal consent is not required. IRB waived off the need to take consent for the study.

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Bothra, S.J., Bhandari, P., Agrawal, N. et al. Extranodal NK-T Cell Lymphoma, Nasal Type: Retrospective Analysis of Real-World Data. Indian J Hematol Blood Transfus 36, 260–266 (2020). https://doi.org/10.1007/s12288-019-01195-6

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