Rare lymphoma (ENKL) has an improved outcome following RT-DeVIC treatment: data supports new prognostic marker

Yamaguchi, M. from the Mie University Graduate School of Medicine,  and colleagues reported the results of a multicenter, retrospective study into the treatment and outcomes of 383 patients newly diagnosed with extranodal natural killer/T-cell lymphoma, nasal type (ENKL), in Japan during the period 2000-2013. This was conducted as part of the NKEA project and was published in the Journal of Clinical Oncology in October 2016.

  • RT-DeVIC accounted for 66% of treatment regimen for ENKL in Japan.
  • For localized ENKL OS = 72% and PFS = 61% with a median follow up period of 5.6 years.
  • For localized ENKL in clinical practice CR = 82% and ORR= 89%
  • All toxicities were resolved and time limited.
  • Elevated sIL-2R levels were an independent prognostic marker for worse survival following RT-DeVIC treatment.  
  • Localized ENKL patients with elevated sIL-2R levels had 5 year OS and PFS rates of 54% and 44% respectively following RT-DeVIC treatment.

The results of this retrospective NKEA study demonstrated that RT-DeVIC treatment of localized ENKL provides a favorable OS in clinical practice. A potential new prognostic marker for ENKL has been identified in sIL-2R, which has been shown to be associated with poor prognosis ENKL. More work will need to be done to further confirm its clinical relevance.


Treatments and Outcomes of Patients With Extranodal Natural Killer/T-Cell Lymphoma Diagnosed Between 2000 and 2013: A Cooperative Study in Japan

Motoko Yamaguchi et al.

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 firstline 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 the 150 patients who received RT-DeVIC in clinical practice were 72% (95% CI, 63% to 78%) and 61% (95% CI, 52% to 69%), respectively. Toxicities of RT-DeVIC were comparable to those in a previous trial. Multivariate analysis in patients with localized ENKL who received RT-DeVIC identified elevated soluble interleukin-2 receptor as an independent predictive factor for worse OS and progression-free survival (adjusted hazard ratios, 2.28 and 2.46; 95% CI, 1.24 to 4.23 and 1.42 to 4.28; P = .008 and .0014, respectively).

Conclusion: Favorable OS in response to new treatments was demonstrated in a large number of patients. Improved treatment approaches are needed for localized ENKL exhibiting elevated pretreatment soluble interleukin-2 receptor.

  1. Yamaguchi, M. et al. Treatments and Outcomes of Patients With Extranodal Natural Killer/T-Cell Lymphoma Diagnosed Between 2000 and 2013: A Cooperative Study in Japan. Journal of Clinical Oncology. 2016 Oct. doi: 10.1200/JCO.2016.68.1619.


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