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Mantle cell lymphoma

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 2023; 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 European Society of Medical Oncology (ESMO 2018; 2017).
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Guidelines

1.Classification and risk stratification

Prognostic scores: as per BSH 2023 guidelines, use the MIPI or MIPI-C for pre-treatment baseline risk stratification.
A
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2.Diagnostic investigations

Initial evaluation: 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

3.Diagnostic procedures

Biopsy: as per BSH 2023 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

4.Medical management

General principles: 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

5.Therapeutic procedures

Stem cell transplantation: as per BSH 2023 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

6.Specific circumstances

Elderly patients, geriatric assessment, BSH: 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

7.Patient education

Fertility counseling: offer fertility counseling or preservation if appropriate in patients with MCL.
B

8.Follow-up and surveillance

Assessment of treatment response
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)