Results from the Belgian mantle cell lymphoma registry

Abstract

Introduction: Mantle cell lymphoma is a B-cell non-Hodgkin's lymphoma characterized by a t (11;14), resulting in overexpression of cyclin D1. Conventional chemotherapy obtains frequent (but short) remissions, leading to a poor median overall survival (OS) of 3-5 years. To obtain more information about the prevalence and current treatment of Mantle cell lymphoma (MCL) in Belgium, we collected data in a Belgian registry of MCL. Materials and methods: All Belgian MCL patients, t(11;14) and/or cyclin D1 positive, seen in hematology departments over a one-year period (April 2013-March 2014) were included. Data about patient characteristics, histology, treatment lines, and response were compiled and retrospectively analyzed. Results: Four hundred and four patients were included with a median age at diagnosis of 64 years (range 23-96 years) and a male predominance (72%). For 2013, we calculated a prevalence of at least 36.2 per million and an incidence of at least 7.0 per million in the Belgian population. Characteristics at diagnosis involved lymphadenopathy (82%), splenomegaly (44%), B-symptoms (39%), and hepatomegaly (10%). Bone marrow invasion was present at diagnosis in 77%. Stage at diagnosis was advanced in the majority of cases. The median number of treatment lines was 1. Type of first line treatment included a combination of anthracyclin and cytarabinebased regimen (34%), anthracyclin (39%), and other. Rituximab was used in 88% of first line treatments. In 44% first line treatment was followed by autologous stem cell transplantation. Conclusion: The analysis of this Belgian MCL registry provides insight in the epidemiology, demographics, and current treatment of our Belgian MCL population.

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

“…Covering the full spectrum of MCL diagnoses, our contemporary real‐world data illustrate the underlying heterogeneity of the disease and its treatment. With a median diagnostic age of 74·0 years, our patient population is, on average, 2–5 years older than other published series (Herrmann et al , ; Abrahamsson et al , , ; Epperla et al , ; Vergote et al , ). This most likely reflects the fact that all patients are identified at the point of diagnosis via a centralised diagnostic laboratory rather than retrospectively from medical records, thereby ensuring all patients are captured regardless of treatment intent; including those managed palliatively from the outset.…”

“…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 , ).…”

Nationwide Assessment of Patient Trajectories in Mantle Cell Lymphoma: The Swedish MCLcomplete Project

“…In this cohort, single modality radiotherapy was given to 27% of patients with stage I-II disease, with favorable results, a median PFS 2.8 years and median OS 11.1 years, indicating that this may be a feasible approach for this population and an approach that may delay the need of systemic treatment. During recent years, several datasets on real-world data on MCL have been reported, from different populations, including data from the US, 17 Belgium, 18 and Japan. 19 As in the Japanese cohort, BR was the most frequently used regimen in our dataset in first, second, and third-line, reflecting that the majority of MCL patients belong to the elderly population.…”

“…During recent years, several datasets on real-world data on MCL have been reported, from different populations, including data from the US, 17 Belgium, 18 and Japan. 19 As in the Japanese cohort, BR was the most frequently used regimen in our dataset in first, second, and third-line, reflecting that the majority of MCL patients belong to the elderly population.…”

Linfoma de células do manto na cavidade oral e região maxilofacial

“…It accounted for approximately 3,320 new cases diagnosed in Western countries in 2016, representing 4% of all lymphoid malignancies in the US and 7-9% in Europe. 1,2 Elderly males are the most commonly affected patients, with lymph nodes being the most affected location.…”