Updates in the treatment of mantle cell lymphoma: A Canadian expert framework

Authors

  • Diego Villa, MD, MPH, FRCPC
  • Roopesh Kansara, MD, FRCPC
  • Christopher Lemieux, MD, FRCPC
  • John Kuruvilla, MD, FRCPC

DOI:

https://doi.org/10.58931/cht.2022.1S1227

Abstract

Mantle cell lymphoma (MCL) is a B cell Non-Hodgkin Lymphoma that develops in the mantle zone of the lymph node. It is more common in men and is usually diagnosed at an advanced stage with involvement of lymph nodes, bone marrow, and potentially the gastrointestinal tract. MCL accounts for 5–10% of all new NHL cases per year in Canada, which is estimated at 11,400 for 2022. While most patients respond to initial treatment, relapses occur early and MCL generally shows a variable response to subsequent treatments, often with limited duration of benefit.

Two main subtypes of MCL can be distinguished that arise from in situ MCL lesions. The most common subtype, classic MCL, arises from these cells with limited or no immunoglobulin heavy chain variable region (IGHV) mutations. Cells from this subtype express SOX11, are genetically unstable, and have naïve B cell-like characteristics. Classic MCL is more often nodal and extranodal and may eventually progress to aggressive blastoid or pleomorphic MCL. The other subtype, leukemic non-nodal MCL, arises from cells that have undergone IGHV somatic hypermutations, do not express SOX11, and exhibit characteristics of memory B cells. This subtype can have an indolent clinical behavior for a long time, often several years, but frequently acquires TP53 and other mutations and progresses to a more aggressive subtype.

Treatment options have expanded significantly over the past decades, with improvements in both overall survival (OS) and progression-free survival (PFS) compared to earlier treatment eras. This Canadian expert framework aims to discuss the management considerations for patients with MCL, and will present both front-line treatment options as well as those for relapsed and refractory disease.

Author Biographies

Diego Villa, MD, MPH, FRCPC

Dr. Diego Villa is Clinical Associate Professor at the University of British Columbia and a medical oncologist at the BC Cancer – Vancouver Cancer Center. He is involved in the care of patients with lymphoid malignancies and breast cancer. His research interests include the management of transformed indolent lymphomas, management of mantle cell lymphoma, primary and secondary CNS lymphomas, and the role of PET/CT in aggressive lymphoma. Dr. Villa has ongoing research collaborations with Canadian, American, European, and Australian lymphoma groups. He is also the local principal investigator for various international lymphoma and breast cancer clinical trials open in Vancouver. He actively participates in the teaching of medical students, residents, and fellows at BC Cancer and VGH, and is the faculty coordinator for the medical oncology rotation at BC Cancer for the UBC Internal Medicine Residency Program. Dr. Villa is a member of the UBC Medical Oncology Residency Training Program committee and its Competence by Design subcommittee.

Roopesh Kansara, MD, FRCPC

Dr. Roopesh Kansara is a staff Hematologist within the Department of Internal Medicine, University of Manitoba. He is an Assistant Professor and is also the Program Director for Adult Hematology subspecialty program at the University of Manitoba. He pursued his Medicine and Hematology subspecialty training at the University of Manitoba followed by further training in lymphoproliferative disorders at the British Columbia Cancer Agency (Vancouver). He treats both malignant and benign hematologic disorders but has a special focus on lymphoproliferative neoplasms, especially CNS B-cell lymphomas.

Christopher Lemieux, MD, FRCPC

Dr. Christopher Lemieux is a Hematologist at the CHU de Québec-Université Laval. He completed specialized training in transplantation and cell therapy at Stanford University in California, for which he received the Detweiler scholarship from the Royal College of Physicians and Surgeons of Canada and the Stephen Couban price of the Canadian Hematology Society. With this experience, he is currently director of the CAR-T program at the CHU de Québec-Université Laval and is a member of the immunocellular therapy network in Québec. He is a Clinical Associate Professor of Medicine and director of the Hematology fellowship program at Université Laval.

John Kuruvilla, MD, FRCPC

Dr. John Kuruvilla is a Professor of Medicine at the University of Toronto and a hematologist in the Division of Medical Oncology and Hematology at the Princess Margaret Cancer Centre in Toronto. He is a member of the Lymphoma, Autologous Transplant and Immune Effector Cell Therapy programs. Dr. Kuruvilla’s research interest is the development of novel therapeutics in lymphoid malignancies and incorporating translational research into clinical trials. He is the Lymphoma Co-Chair for the Canadian Cancer Trials Group (CCTG) as well as the Chair of the Scientific Advisory Board of Lymphoma Canada.

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2022-12-14

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1.
Villa D, Kansara R, Lemieux C, Kuruvilla J. Updates in the treatment of mantle cell lymphoma: A Canadian expert framework. Can Hematol Today [Internet]. 2022 Dec. 14 [cited 2024 Apr. 15];1(S12):2–11. Available from: https://canadianhematologytoday.com/article/view/1-s12-villa_et_al