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.

Highlights:

• 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.

Conclusions:

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.

 Abstract:

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.