ESMO E-Learning: Mantle Cell Lymphoma

Learning Objectives

  • To understand the biology of mantle cell lymphoma (MCL)
  • To understand the diagnostic and prognostic factors in MCL
  • To choose the most appropriate first-line treatment for different subsets of patients with MCL
  • To learn about molecular targeted substances and therapy concepts at relapse and refractory disease
Title Duration Content CME Points CME Test
Mantle Cell Lymphoma 51 min. 43 slides 1 Take Test
weiglein-tobias
Tobias Weiglein
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dreyling-martin-heinz
Martin Heinz Dreyling
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Mantle cell lymphoma (MCL) accounts for approximately 6% of all Non-Hodgkin lymphoma cases (NHL). Median age at diagnosis is 60-70 years with male predominance. It is characterised by a very frequent extranodal disease manifestation. MCL has a dismal prognosis with a shorter median overall survival (OS) rate compared to all other  NHL. 

In this E-learning module, the authors provide a state-of-the-art in the biology, diagnostic and prognostic factors, first-line and the treatment concepts in relapsed/refractory MCL, as well as a future prospect. 

In terms of first-line treatment, Rituximab(R)-chemotherapy is standard of care as induction therapy. In younger, fit patients, induction-regimens containing high-dose cytarabin seem to further improve outcome. High-dose chemotherapy, followed by autologous stem cell transplantation (ASCT) followed by rituximab maintenance is the current standard, generally providing high responses and prolonged progression-free survival. R-hyper-CVAD, followed by a consolidation with BEAM and ASCT is an alternative dose intensified approach, but is hampered by higher toxicity. Rituximab maintenance every 2 months for 3 years after ASCT improves OS. In patients with an indolent course of disease and low tumour burden, the authors underline the option of the watch and wait strategy. 

In elderly patients, with compromised organ function and performance score, conventional immuno-chemotherapy is still the first treatment choice. Fludarabin-containing regimens are inferior to R-CHOP in terms of OS. Rituximab maintenance therapy every 2 months following R-CHOP chemotherapy significantly improves progression-free survival (PFS). Bortezomib in combination with R-CAP shows better PFS compared to R-CHOP with significantly more haematological toxicity and represents a good option for aggressive MCL in elderly patients not eligible for ASCT. Bendamustin in combination with rituximab achieves at least comparable outcomes as R-CHOP, but with less side effects (especially alopecia) and is therefore preferred in elderly patients. In very frail patients, rituximab monotherapy or rituximab and oral chlorambucil is an effective and well tolerated option, especially in low risk or rather indolent cases. 

In relapse, immuno-chemotherapy is one standard of care with regimens selected depending on patient fitness and age, comorbidities, earlier therapeutic lines and duration of response. Temsirolimus achieves higher response rates when compared to mono-chemotherapy, but may be combined with chemotherapy to achieve higher efficacy. In comparison, ibrutinib, an oral BTK-inhibitor, results in significantly higher response rates and longer median PFS. Lenalidomide also increases ORR and PFS compared to monochemotherapy in higher relapse. Bortezomib shows promising activity especially in combination with chemotherapy. Rituximab maintenance after salvage immuno-chemotherapy leads to improved PFS. If high-dose chemotherapy and ASCT has not been performed as first-line therapy, eligible patients should be offered that option. Young fit patients relapsing after high-dose chemotherapy and ASCT should be offered an allogenic transplantation if a donor is available.

Last update: 30 July 2018

Dr Weiglein has reported no conflict of interest.

Prof Dreyling has reported Speaker’s honoraria from Bayer, Celgene, Gilead, Janssen and Roche. Scientific Advisory Board for Bayer, Celgene, Gilead, Janssen, Multipharma, Roche and Sandoz. Institutional and research support from Celgene, Janssen, Multipharma and Roche.