Real-world data on prognostic factors and treatment in peripheral T-cell lymphomas: a study from the Swedish Lymphoma Registry

Abstract

Key Points• Population-based data show a favorable outcome with upfront autologous stem cell transplantation in PTCL.• The addition of etoposide to CHOP was associated with favorable PFS in patients #60 years with PTCL.Peripheral T-cell lymphomas (PTCLs) are rare lymphomas with mostly poor outcome with current treatment. The addition of etoposide to cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) and upfront consolidation with autologous stem cell transplantation (auto-SCT) have shown promising results but have never been tested in randomized trials. As a complement to retrospective analyses of clinical trials, we aimed at analyzing prognostic factors and outcome in an unselected, population-based cohort. Through the Swedish Lymphoma Registry, we identified 755 PTCL patients diagnosed during a 10-year period. In addition to International Prognostic Index factors, male gender was associated with an adverse overall survival (OS) (hazard ratio [HR], 1.28; P 5 .011) and progression-free survival (PFS) (HR, 1.26; P 5 .014). In an intention-to-treat analysis in 252 nodal PTCL and enteropathy-associated T-cell lymphoma patients (excluding anaplastic lymphoma kinase-positive anaplastic large cell lymphoma), upfront auto-SCT was associated with a superior OS (HR, 0.58; P 5 .004) and PFS (HR, 0.56; P 5 .002) compared with patients treated without auto-SCT. The addition of etoposide to CHOP resulted in superior PFS in patients £60 years (HR, 0.49; P 5 .008). This study is the largest population-based PTCL cohort reported so far and provides important information on outcome in PTCL outside the setting of clinical trials. (Blood. 2014;124(10):1570-1577

Autologous stem cell transplantation in first complete remission may not extend progression‐free survival in patients with peripheral T cell lymphomas

“…However, these results should be interpreted with caution as 64% (18/28) of the nontransplanted patients were excluded from transplant because of progressive disease. Likewise, a recent registry based retrospective study from Sweden [31] reported a 5-year PFS of 41% in patients who were planned for upfront ASCT versus 20% in patients who were not. However, the patients who were planned for upfront ASCT were significantly younger when compared to the patients that were not.…”

Redefining the role of etoposide in first-line treatment of peripheral T-cell lymphoma

“…This finding goes against the current guidelines but is in agreement with a previous large-cohort study by the Swedish group. 5 Because Ellin et al 5 suggested etoposide might have a role in younger patients with PTCL, we examined the effect of etoposide addition in those age ,45 years. As presented in Figure 3, we found that etoposide addition and chemotherapy intensification did not improve survival, even in fit and young patients.…”

“…1,2 Despite the clinical differences between B-and T-cell lymphomas, 3 and despite producing somewhat less-than-satisfactory results, the combination of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) remains the de facto standard in PTCL treatment, because evidence showing other regimens to be superior is lacking. 4,5 Functioning as a phase-sensitive cytocidal drug that specifically affects cells in S or G2, etoposide offers non-cross-resistant cytotoxicity when used in combination with alkylating agents and doxorubicin. Thus, it has been integrated into multiagent protocols used in the primary treatment of various subtypes of malignant lymphomas.…”

Anaplastic large cell lymphoma: pathology, genetics, and clinical aspects

“…Similar results have been reported in an analysis of Swedish registry data on patients with PTCL. 17 Among 118 patients with stage I/II PTCL, including ALCL, 32 cases received consolidative local radiotherapy at a median dose of 40 Gy. A trend of superior survival in patients who underwent irradiation was observed, but no risk reduction associated with the addition of radiotherapy was found by multivariate analysis.…”

“…A male predominance is observed, and the maleto-female ratio is approximately 1.2 to 3.0. 5,[9][10][11][12][13][14][15][16][17] The majority (50-70%) of patients present with stage III-IV advanced disease with peripheral, including mediastinal, and/or abdominal lymphadenopathy. 5,[10][11][12][13][14] Patients often have B symptoms (54-75%), 5,9,10,12,13,15 and extranodal involvement is frequently observed (approximately 60% of cases).…”