What are the NCCN and ESMO treatment guidelines for mantle cell lymphoma (MCL)?

Updated: Mar 15, 2019
  • Author: Muhammad Rashid Abbasi, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Answer

The NCCN and ESMO offer similar treatment recommendations, as follows [8, 35]

  • Chemotherapy followed by involved-site radiation therapy (ISRT), 30-36 Gy, is the preferred treatment option for limited stage I or II (non-bulky) disease, although this presentation is rare

  • For advanced-stage disease in younger patients and selected elderly fit patients, the recommended approach is aggressive induction therapy with a regimen such as hyperCVAD (cyclophosphamide, vincristine, doxorubicin [Adriamycin], dexamethasone alternating with high-dose methotrexate and cytarabine) + rituximab, followed by consolidation therapy consisting of high-dose therapy with autologous stem cell rescue

  • Prophylaxis and monitoring for tumor lysis syndrome should be strongly considered during the induction therapy.

  • In elderly fit patients, less-aggressive treatment regimens, such as R-CHOP (rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone) followed by rituximab maintenance is recommended by both NCCN and ESMO

  • For elderly patients who are not candidates for any of the above chemotherapy regimens, palliative chemotherapy should be considered, using milder chemo-immunotherapy regimens (eg, chlorambucil plus rituximab, bendamustine plus rituximab)

  • For relapsed or refractory disease, recommendations include high-dose therapy with autologous stem cell rescue and second-line agents bendamustine, bortezomib, temsirolimus, ibrutinib or lenalidomide with rituximab; allogeneic stem cell transplantation can be considered in selected patients as part of a second-line consolidation

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