Overview of Mantle Cell Lymphoma and First-Line Treatment Options


Alexey V. Danilov, MD, PhD, provides an overview of the pathophysiology, symptoms, and subtypes of mantle cell lymphoma.


Alexey Danilov, MD, PhD: Hello, and welcome to this OncLive Peer Exchange® entitled “Recent Advances in the Treatment of Mantle Cell Lymphoma.” I’m Alexey Danilov, professor at City of Hope and leader of the lymphoma program. I’m joined today by a panel of experts in MCL [mantle cell lymphoma]. I would like to welcome my esteemed fellow panelists to introduce themselves.

Bijal Shah, MD: Hi, I’m Dr Bijal Shah from the Moffitt Cancer Center, where I focus on mantle cell lymphoma and acute lymphoblastic leukemia.

Matthew J. Matasar, MD: I’m Dr Matthew Matasar. I’m the chief of blood disorders at the Rutgers Cancer Institute of New Jersey.

Boyu Hu, MD: I’m Dr Boyu Hu, part of the lymphoma CLL [chronic lymphocytic leukemia] group at the University of Utah.

Alexey Danilov, MD, PhD: Welcome, everyone, and thank you for joining me. Today we are going to discuss a number of recent updates in the treatment of MCL, with particular focus on updates from 2022. We will discuss the data in the context of guidelines, the treatment landscape, and its impact on clinical practice. Let’s get started on this first topic.

Mantle cell lymphoma is a fairly uncommon subtype of B-cell non-Hodgkin lymphoma with about 3000 to 4000 cases diagnosed each year in the United States. Mantle cell lymphoma is characterized by a translocation of chromosome 11 to chromosome 14, which ultimately results in overexpression of cyclin D1, a protein that is responsible for [cell cycle] regulation. Mantle cell lymphoma has a heterogeneous cause. There are 2 main subtypes: classical mantle cell lymphoma, which is characterized by unmutated IgHV [immunoglobulin heavy chain], and overexpression of SOX11. Within classical mantle cell lymphoma, we also have blastoids and pleomorphic subtypes, which are more aggressive. Meanwhile, leukemic nonnodal MCL is IgHV mutated and doesn’t express SOX11. Patients with mantle cell lymphoma present with B symptoms, like progressive lymphadenopathy, and sometimes cytopenias. About 70% of patients will have bone marrow involvement and, particularly, patients with leukemic nonnodal MCL have splenomegaly.

The risk of MCL is defined by using multiple strategies. MIPI [Mantle Cell Lymphoma International Prognostic Index] score is most commonly used. Ki67 can be added to the MIPI score. A Ki67 index of over 30% is associated with inferior outcomes. In recent years, genetic predictors of MCL outcomes have become important, such as patients who have TP53 aberrations, [who,] unfortunately, have inferior outcomes in mantle cell lymphoma. Complex karyotype has also been identified as something that is associated with inferior outcomes in MCL. Complex karyotype is defined as 3 or more chromosomal abnormalities.

First-line treatment of MCL can be subdivided essentially into aggressive therapy or less aggressive therapy depending on patient presentation and comorbidities. To this day, patients who are eligible for autologous stem cell transplant typically undergo aggressive chemotherapy-based induction, and that could be the Nordic regimen, DHAP [dexamethasone/cytarabine/cisplatin], or some other regimen followed by consolidation with autologous stem cell transplant and rituximab maintenance. Meanwhile, patients who are older and are not good candidates for autologous stem cell therapy would usually be receiving bendamustine-rituximab or another regimen, such as VR-CAP [bortezomib plus rituximab, cyclophosphamide, epirubicin, prednisone]. Lenalidomide-rituximab can also be used in these patients based on NCCN guidelines. Let’s turn our attention to the first case.

Transcript edited for clarity.

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