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Tx Selection and Optimization in MCL

CME

Optimizing Mantle Cell Lymphoma Care: Case-Based Guidance From EU Experts

Physicians: Maximum of 1.00 AMA PRA Category 1 Credit™

Released: May 29, 2026

Expiration: November 28, 2026

Mats Jerkeman
Mats Jerkeman, MD, PhD
CME/CE Information

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MCL: Background

MCL is a clinically heterogenous type of B-cell non-Hodgkin lymphoma (NHL).1,2 It can be indolent, but it can also be very aggressive and refractory to treatment. The incidence of MCL is approximately 5% to 7% of all NHL in the United States and Europe. It is less common in Asian countries. MCL is characterized by t(11;14), which leads to the overexpression of cyclin D1. The median age at diagnosis is approximately 70 years, and most patients diagnosed with MCL are males, accounting for approximately 75% of patients with the disease. Typically, MCL is diagnosed in the advanced stage with extranodal involvement of sites, such as the bone marrow, gastrointestinal tract, and spleen. MCL is often initially responsive to therapy, but in most cases, patients experience multiple treatment relapses. At present, it is unclear if any patient with MCL can be cured of the disease.

MCL: Risk Factors

For patients with MCL, there are several recognized biologic risk factors that are critical for treatment decision-making. Biologic factors associated with a favorable treatment outcome include a low proliferation rate with Ki-67 expression of ≤10% and the presence of a stable karyotype.3 Patients with hypermutated IGHV and SOX-11 negativity have a better prognosis. These factors are associated with the more indolent cases of MCL.

An important high-risk factor is the presence of nonclassical histology, specifically the blastoid and pleomorphic MCL subtypes. A high proliferation rate with Ki-67 expression >30% is also a poor prognostic factor. However, the most important biological high-risk factor associated with poor prognosis is the presence of a TP53 mutation, which predisposes patients for short response to CIT, the conventional standard of care in MCL.

Currently, a critical knowledge gap is identifying how to treat patients with high-risk MCL, especially those harboring a TP53 mutation or p53 protein overexpression, as there is currently no clear solution or consensus. Of importance, the evolution of novel agents and approaches has resulted in significant benefits for the CIT.

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NCCN Guidelines: Updated Diagnostic Workup in MCL

Diagnostic workup to assess whether a patient has MCL includes a physical examination with attention to peripheral lymph nodes and the Waldeyer’s ring.4 The determination of the complete blood count with differential is important because many patients present with lymphocytosis. An essential prognostic factor in MCL is the assessment of lactate dehydrogenase levels, and the diagnostic workup should include radiologic examinations using PET/CT imaging with contrast of diagnostic quality if systemic therapy is planned.

If this is not possible, a CT scan of the neck, thorax, and abdomen can be performed. In some cases, such as for patients with possible limited-stage MCL who may be candidates for radiotherapy only, an endoscopy and a bone marrow biopsy with aspirate and a PET scan are recommended to confirm the presence of localized disease.

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