Mantle cell lymphoma epidemiology: a population-based study in France

Abstract

Only limited population-based data are available on mantle cell lymphoma (MCL), a relatively rare and aggressive mature B cell non-Hodgkin lymphoma (NHL) entity. We conducted an epidemiological study based on the three French registries devoted to haematological malignancies over the period 2002-2006. Main clinical features and management characteristics were collected. Incidence and survival rates were estimated, and independent prognostic factors were analysed. MCL was diagnosed in 135 patients between 2002 and 2006. Seventy-four percent of patients were men. Age-standardised incidence rate of MCL (per 100,000) was 1.1 in men and 0.26 in women. Median age at diagnosis was 72 years (range 30-92). Advanced-stage (III or IV) disease was diagnosed in 81.5 % of patients, and 55 % of them were identified as high risk according to MCL International Prognostic Index (MIPI). Median net survival time was 41 months (95 % confidence interval (CI), 38-62). Main independent prognostic factors were age at diagnosis, performance status and use of rituximab in first-line treatment. Median overall survival was 36 months (95 % CI, 27-40) for high MIPI and 60 months (95 % CI, 35-74) for low/intermediate MIPI patients (p = 0.02). This study confirms that MCL remains an aggressive NHL with a median overall survival less than 4 years and demonstrates that the use of rituximab has modified overall survival duration.

PhaseIItrial of R‐CHOPplus bortezomib induction therapy followed by bortezomib maintenance for newly diagnosed mantle cell lymphoma:SWOGS0601

“…Although the survival of patients with MCL has improved over the last decade, much of this benefit is associated with selected populations of younger patients receiving intensive therapy (Delarue et al, 2013; Geisler et al, 2008; Hermine et al, 2010). Unfortunately, aggressive therapies are not feasible for many patients with MCL, and in population-based studies, where the median age at diagnosis is 70 or older, the median overall survival (OS) remains 3–5 years (Abrahamsson et al, 2014; Leux et al, 2014; van de Schans et al, 2010). The regimen of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) is a standard induction therapy for MCL that is associated with high response rates, but remissions are not durable, with a median progression-free survival (PFS) of 14 to 22 months (Howard et al, 2002; Lenz et al, 2005; Robak et al, 2015; Rummel et al, 2013).…”

Impact of novel therapies for mantle cell lymphoma in the real world setting: a report from the UK's Haematological Malignancy Research Network (HMRN)

“…First recognized as an official entity in 1994 (Harris et al , ), mantle cell lymphoma (MCL) is a relatively rare B‐cell malignancy characterized by diverse patient pathways and a generally poor prognosis (Martin et al , ). With a median diagnostic age of around 70 years, MCL occurs 2‐3 times more frequently in men than women (Abrahamsson et al , ; Leux et al , ; Smith et al , ; Vergote et al , ); and although a small subset of patients present with localized/indolent disease that can be treated with radiotherapy and/or managed by watch and wait (W&W), the majority present with advanced disease that requires chemotherapy (McKay et al , ; Dreyling et al , ; National Comprehensive Cancer Network, ). Unfortunately, however, even though many patients respond well to first‐line treatment, remission is generally short‐lived, response to second and subsequent lines is poor, and survival is lower than most other lymphoma subtypes (Marcos‐Gragera et al , ; Chandran et al , ; Smith et al , ) with 5‐year overall survival estimates ranging from 30% to 50% in the general patient population (Marcos‐Gragera et al , ; Abrahamsson et al , ; Smith et al , ).…”

Walking a tightrope: clinical use of ibrutinib in mantle cell lymphoma in the elderly

“…2,3 More recent real-world studies indicate that the median age of patients with MCL may be older than 70 years. [4][5][6] Therefore, MCL is considered a disease of the elderly, and a significant subset of patients displays reduced performance status at diagnosis. Overall, MCL has a poor prognosis with 4 to 5 years of median survival, 7 and elderly patients (age 65 to 70 years or older) have a worse outcome compared with younger patients, mostly because of increased treatment-related toxicity.…”