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EHA–EU MCL network guidelines for diagnosis and treatment of mantle cell lymphoma

Jerkeman, Mats LU ; Aurer, Igor ; Campo, Elias ; Cheah, Chan Y. ; Clark, Jonathan ; Doorduijn, Jeanette ; Eyre, Toby A. ; Fehr, Martin ; Giné, Eva and Gomes da Silva, Maria , et al. (2025) In HemaSphere 9(10).
Abstract

Mantle cell lymphoma (MCL) is a relatively rare B-cell lymphoma subtype, with a higher incidence among males and a median age of 70 years at diagnosis. MCL is characterized by clinically diverse behavior, from indolent disease to extremely aggressive, related to the presence of biological risk factors such as proliferation rate and TP53 mutations. Most often, patients present with disseminated disease, necessitating systemic treatment. Immunochemotherapy has historically been the mainstay of treatment, but recent data indicate that addition of novel agents, especially covalent Bruton tyrosine kinase inhibitors (cBTKi), may substantially improve outcome in younger and older patients, although a curative approach remains to be shown. In... (More)

Mantle cell lymphoma (MCL) is a relatively rare B-cell lymphoma subtype, with a higher incidence among males and a median age of 70 years at diagnosis. MCL is characterized by clinically diverse behavior, from indolent disease to extremely aggressive, related to the presence of biological risk factors such as proliferation rate and TP53 mutations. Most often, patients present with disseminated disease, necessitating systemic treatment. Immunochemotherapy has historically been the mainstay of treatment, but recent data indicate that addition of novel agents, especially covalent Bruton tyrosine kinase inhibitors (cBTKi), may substantially improve outcome in younger and older patients, although a curative approach remains to be shown. In elderly patients, the standard of care is still immuno-chemotherapy such as rituximab-bendamustine, although this may be challenged by non-chemotherapeutic options, such as rituximab plus cBTKi. For patients with relapsed or refractory disease, treatment options are developing rapidly, including CAR-T cell therapy, novel BTK targeting agents, BCL2 inhibitors, and T-cell engagers. In this clinical practice guideline, we present current evidence-based recommendations for diagnosis, staging, treatment, and follow-up of MCL.

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author collaboration
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
HemaSphere
volume
9
issue
10
article number
e70233
publisher
Wolters Kluwer
external identifiers
  • scopus:105021030165
  • pmid:41132246
ISSN
2572-9241
DOI
10.1002/hem3.70233
language
English
LU publication?
yes
id
5a44b5fd-82ad-4784-b4bb-5bb8e5310c04
date added to LUP
2025-12-19 10:44:15
date last changed
2025-12-20 03:00:24
@article{5a44b5fd-82ad-4784-b4bb-5bb8e5310c04,
  abstract     = {{<p>Mantle cell lymphoma (MCL) is a relatively rare B-cell lymphoma subtype, with a higher incidence among males and a median age of 70 years at diagnosis. MCL is characterized by clinically diverse behavior, from indolent disease to extremely aggressive, related to the presence of biological risk factors such as proliferation rate and TP53 mutations. Most often, patients present with disseminated disease, necessitating systemic treatment. Immunochemotherapy has historically been the mainstay of treatment, but recent data indicate that addition of novel agents, especially covalent Bruton tyrosine kinase inhibitors (cBTKi), may substantially improve outcome in younger and older patients, although a curative approach remains to be shown. In elderly patients, the standard of care is still immuno-chemotherapy such as rituximab-bendamustine, although this may be challenged by non-chemotherapeutic options, such as rituximab plus cBTKi. For patients with relapsed or refractory disease, treatment options are developing rapidly, including CAR-T cell therapy, novel BTK targeting agents, BCL2 inhibitors, and T-cell engagers. In this clinical practice guideline, we present current evidence-based recommendations for diagnosis, staging, treatment, and follow-up of MCL.</p>}},
  author       = {{Jerkeman, Mats and Aurer, Igor and Campo, Elias and Cheah, Chan Y. and Clark, Jonathan and Doorduijn, Jeanette and Eyre, Toby A. and Fehr, Martin and Giné, Eva and Gomes da Silva, Maria and Klener, Pavel and Ladetto, Marco and Ribrag, Vincent and Shpilberg, Ofer and Walewski, Jan and Dreyling, Martin}},
  issn         = {{2572-9241}},
  language     = {{eng}},
  number       = {{10}},
  publisher    = {{Wolters Kluwer}},
  series       = {{HemaSphere}},
  title        = {{EHA–EU MCL network guidelines for diagnosis and treatment of mantle cell lymphoma}},
  url          = {{http://dx.doi.org/10.1002/hem3.70233}},
  doi          = {{10.1002/hem3.70233}},
  volume       = {{9}},
  year         = {{2025}},
}