Abstract
Mantle cell lymphoma (MCL) is a B-cell non-Hodgkin lymphoma with historically poor long-term survival compared with other B-cell malignancies. Treatment strategies for this disease are variable and dependent on symptoms and patient fitness. Despite recent advances, MCL remains incurable and patients with high-risk disease have particularly poor outcomes. This review focuses on recent developments that enhance our understanding of the biology of MCL and new treatment approaches that have led to substantial improvements in clinical outcomes. We will outline induction immuno-chemotherapy and maintenance strategies in transplant-eligible patients. In addition, effective strategies for patients unfit for intensive induction will be discussed, with a particular focus on novel molecular therapies with activity in MCL. Lastly, a number of ongoing clinical trials will be presented; the data from these trials are anticipated to redefine standards of care in the near future.
Highlights
Mantle cell lymphoma (MCL) is an aggressive mature B-cell non-Hodgkin lymphoma (B-NHL) with historically poor long-term survival
We will discuss the major breakthroughs in MCL care in the past 5 years, including recent phase III clinical data that reinforce the use of high-dose cytarabine induction immunochemotherapy in fit patients
There are data from randomized studies supporting the use of rituximab maintenance in MCL post-autologous stem cell transplant (ASCT)
Summary
Mantle cell lymphoma (MCL) is an aggressive mature B-cell non-Hodgkin lymphoma (B-NHL) with historically poor long-term survival. The MCL Younger trial conducted by the European MCL Network provided phase III data to support cytarabine as the key agent in MCL induction[14] This trial included patients aged 65 years or younger with stage II–IV MCL randomized to six cycles of R-CHOP (non-cytarabine group) versus alternating R-CHOP and R-DHAP for six cycles (cytarabine group). Among the 5% of patients with a high combined MIPI and Ki-67 expression score treated in the European MCL Network Younger trial, median overall survival was approximately 2 years[8] Each of these patient subsets still perform poorly, even with modern cytarabine-containing regimens and/or ASCT, suggesting that novel treatment strategies are needed for these groups.
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