The present guidelines cover the systemic subtypes of primary nodal and primary extranodal peripheral T-cell lymphomas (PTCLs). ESMO guidelines for primary cutaneous T-cell lymphomas are published separately [1]. Primary leukaemic PTCL subtypes (i.e. T-cell prolymphocytic leukaemia, T-cell large granular lymphocytic leukaemia, adult T-cell leukaemia/lymphoma and aggressive NK-cell leukaemia) are not covered by the present guidelines. Primary nodal PTCLs include PTCL-not otherwise specified (PTCL-NOS), anaplastic large-cell lymphoma (ALCL), both fusion protein ALCL anaplastic lymphoma kinase positive (ALCL ALK+) and ALCL anaplastic lymphoma kinase negative (ALCL ALK−), and angioimmunoblastic T-cell lymphoma (AITL). The primary extranodal PTCL subtypes covered by the present guidelines comprise enteropathy-associated T-cell lymphoma (EATL), extranodal natural killer/T-cell lymphoma (ENKTCL), and hepatosplenic T-cell lymphoma (HSTCL). † Approved by the ESMO Guidelines Committee: June 2015.

The present study aimed to analyse the treatment outcome of four cycles of CHOP (cyclophosphamide-vincristine-doxorubicin-prednisolone) followed by involved field radiation therapy (IF RT) for the treatment of stage I-II nasal natural killer (NK)/T-cell lymphoma. From March 1995 to December 1999, 17 patients (median age 41 years; range 30-66) with localized nasal NK/T-cell lymphoma were enrolled. B symptoms were noted in five patients (31%). Sixteen of seventeen patients (94%) were of low risk when classified according to the International Prognostic Index (IPI). The treatment plan consisted of four cycles of CHOP chemotherapy followed by IF RT of 45 Gy. Two patients received radiation during the first or second cycle of CHOP because of bleeding from the primary tumour site. Both patients achieved complete responses (CRs). In the remaining 15 patients, after 4 cycles of CHOP, 6 CRs and 3 partial responses (PRs) were achieved (53% of response rate). IF RT was given to six patients (four in CR, one in PR and one in PD), and all six patients achieved CR. Overall, CR was achieved in 10 of 17 patients (58%). The planned sequential chemoradiotherapy was completed in only 6 of 17 patients (35%) because of the progression during chemotherapy. None of the patients who achieved CR experienced relapse of lymphoma during follow-up. The estimated overall three-year survival rate was 59%. In univariate analysis, B symptoms and stage were significant prognostic factors for response and overall survival (P < 0.05). The present study suggests that four cycles of CHOP followed by IF RT is not satisfactory for treating patients with localized nasal NK/T-cell lymphoma, and that further exploration for improved therapy is needed.

In stage I/II NK/T-cell lymphomas, when effective chemotherapeutic regimens were used, CCRT and sequential CT + RT gave similar outcome.

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