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Mantle cell lymphoma

What's new

Updated 2023 BSH guidelines for the diagnosis and management of mantle cell lymphoma.

Guidelines

Key sources

The following summarized guidelines for the evaluation and management of mantle cell lymphoma are prepared by our editorial team based on guidelines from the British Society for Haematology (BSH 2024,2018), the American Society for Clinical Pathology (ASCP/CAP 2021), the European Society for Blood and Marrow Transplantation (EBMT/CIBMTR/ASTCT 2021), the American Society of Blood and Marrow Transplantation (ASBMT/EBMT/CIBMTR 2019), and the ...
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Classification and risk stratification

Prognostic scores: as per BSH 2024 guidelines, use the MIPI or MIPI-C for pre-treatment baseline risk stratification.
A
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Diagnostic investigations

Initial evaluation: as per BSH 2024 guidelines, obtain clinical assessment, blood tests including a CBC, blood film, routine biochemistry (including uric acid and LDH), and screening for HBV, HCV, and HIV infections.
B

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  • Imaging for staging

Diagnostic procedures

Biopsy: as per BSH 2024 guidelines, perform bone marrow biopsy with or without aspiration if required for formal staging or to evaluate cytopenias before treatment, as PET/CT has low sensitivity to detect bone marrow involvement.
B

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  • FNA

  • Ancillary testing

  • Lumbar puncture

Medical management

General principles: as per BSH 2018 guidelines, take into account the following when deciding on the treatment of patients with MCL:
clinical presentation (with recognition of the indolent form)
proliferation index
clinical risk scores (such as the sMIPI and MIPI-C)
performance status.
B

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  • Induction therapy

  • Maintenance therapy

Therapeutic procedures

Stem cell transplantation: as per BSH 2024 guidelines, consider offering allo-SCT in fit patients with an appropriate donor following immunochemotherapy, covalent Bruton TKI, and CAR T-cell therapy failure.
C

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  • CAR T-cell therapy

Specific circumstances

Elderly patients, geriatric assessment: as per BSH 2024 guidelines, consider obtaining a formal frailty assessment in potentially frail patients.
C

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  • Elderly patients (diagnostic evaluation)

  • Elderly patients (chemoimmunotherapy)

  • Elderly patients (stem cell transplantation)

  • Elderly patients (assessment of QoL)

  • Patients with indolent MCL

  • Patients with CNS MCL

Patient education

Fertility counseling: as per BSH 2024 guidelines, offer fertility counseling or preservation if appropriate in patients with MCL.
B

Follow-up and surveillance

Assessment of treatment response
As per BSH 2018 guidelines:
Obtain conventional CT for the assessment of treatment response.
B
Do not obtain 18F-FDG-PET or evaluation of minimal residual disease status for the assessment of treatment response outside a clinical trial.
D

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  • Management of relapse (systemic therapy)

  • Management of relapse (CAR T-cell therapy)

  • Management of relapse (allo-SCT)