Mantle Cell Lymphoma: From Biology to Therapy

Dear Colleagues,

Mantle cell lymphoma (MCL) is a heterogeneous disease comprising around 2.5–6% of B-cell non-Hodgkin lymphoma cases. The primary genetic alteration in MCL is chromosomal translocation (11;14) which leads to CyclinD1 overexpression and uncontrolled cell proliferation. There are several variants of MCL including leukemic non-nodal, classic, blastoid, and pleomorphic, which have varying biological characteristics that lead to different disease courses and long-term outcomes. There are several independent prognostic factors in MCL including the MIPI, MIPI-b, Ki-67%>30%, TP53 mutation, SOX11 expression, complex cytogenetics, and MCL35 assay with several other factors/risk scores currently under evaluation.

The survival of patients with MCL has significantly improved over the past decade due to better understanding of the disease biology and the advent of targeted therapies. Small-molecule inhibitors such as proteasome inhibitors, immunomodulators, BTK inhibitors, and BCL2 inhibitors either alone or in combination have changed the treatment landscape and outcomes regarding this disease. Several other antibody–drug conjugates, cell cycles, and intracellular signaling inhibitors are currently under evaluation. More recently, CD19-directed CART cell therapy and bispecific antibodies have ushered an era of cellular therapies in MCL with remarkable outcomes, even in high-risk subsets.

In this issue, we will focus on the advancements made in the field of MCL starting with biology and discussing the current and emerging therapies in light of these advancements.   

Dr. Narendranath Epperla
Guest Editor

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• MCL
• biology
• BTK inhibitors
• BCL2 inhibitors
• CART
• bispecific antibodies