Real world data on primary treatment for mantle cell lymphoma: a Nordic Lymphoma Group observational study

Abstract

• Rituximab and autologous stem cell transplantation are both independently associated with improved overall survival in mantle cell lymphoma.• Male gender is an independent negative prognostic factor in mantle cell lymphoma.There is consensus that young patients with mantle cell lymphoma (MCL) should receive intensive immunochemotherapy regimens, but optimal treatment of elderly patients as well for as patients with limited or indolent disease is not defined. Our aim was to evaluate and compare outcome in relation to prognostic factors and first-line treatment in patients with MCL in a population-based data set. Data were collected from the Swedish and Danish Lymphoma Registries from the period of 2000 to 2011. A total of 1389 patients were diagnosed with MCL. During this period, age-standardized incidence MCL increased, most prominently among males. Furthermore, male gender was associated with inferior overall survival (OS) in multivariate analysis (hazard ratio [HR] 5 1.36; P 5 .002). Forty-three (3.6%) patients with stage I-II disease received radiotherapy with curative intent, showing a 3-year OS of 93%. Twenty-nine (2.4%) patients followed a watch-and-wait approach and showed a 3-year OS of 79.8%. Among patients receiving systemic treatment, rituximab (n 5 766; HR 5 0.66; P 5 .001) and autologous stem cell transplant (n 5 273; HR 5 0.55; P 5 .004) were independently associated with improved OS in multivariate analysis. Hence, by a population-based approach, we were able to provide novel data on prognostic factors and primary treatment of MCL, applicable to routine clinical practice. (Blood. 2014;124(8):1288-1295

TP53 mutations identify younger mantle cell lymphoma patients who do not benefit from intensive chemoimmunotherapy

“…Historically, MCL was associated with a dismal outcome, with a median overall survival (OS) of only 3 to 5 years 1,2 ; however, during the past decades, the outcome, especially for younger patients, has improved substantially by an intensified frontline regimen including cytarabine, rituximab, and consolidation with high-dose therapy and autologous stem-cell transplant (ASCT). 1,3,4 Hence, in the recent long-term follow-up of such a regimen, the Nordic MCL2 trial, we observed a median OS and progression-free survival (PFS) of 12.7 and 8.5 years, respectively.…”

Mantle Cell Lymphoma: Current Concepts

“…Retrospective data suggests that use of radiotherapy with or without chemotherapy engendered significantly better progression-free survival (PFS) at 5 years (73% vs. 13%; p=0.001) with a trend towards overall survival benefit, when compared to patients who did not receive radiation [14]. Radiotherapy as a primary treatment for stage 1-2 MCL patients analyzed retrospectively showed curative results for 3.6% of patients, with 3-year OS of 93% [15].…”

The potential for chemotherapy-free strategies in mantle cell lymphoma

“…These factors likely contribute to the discordance between outcomes described in an optimized clinical trial population, which have improved significantly over the past decade, and real-world outcomes, which remain disappointing. 3 Where is there a role for nonchemotherapy treatment of MCL?…”

“…11 Nonetheless, the most effective immunochemotherapy regimens are restricted to young, healthy patients who account for ,25% of all patients. 3 Cytotoxic chemotherapy is associated with well-documented toxicities. In addition to usually modest nuisance-type side effects that can sometimes be more significant, such as alopecia, nausea, and fatigue, chemotherapy can cause significant hematological toxicities, resulting in neutropenic fever and other opportunistic infections.…”

“…In some cases, these nonchemotherapy drugs appear to be active where traditional cytotoxic chemotherapy might fail, and in many cases the side-effect profile is distinct from chemotherapy. Despite the failure of any of these drugs to improve OS in randomized trials, their introduction corresponds with better outcomes in population studies [3][4][5] suggesting that the availability of new options that can be used sequentially may be extending survival. Increasingly, clinicians, patients, and other interested parties are raising the possibility of a future without chemotherapy.…”