How is mantle cell lymphoma (MCL) treated?

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

Treatment of mantle cell lymphoma (MCL) remains a difficult problem because of the lack of reliably curative treatments and the paucity of prospective clinical trials. [6] Although 50-90% of these patients respond to combination chemotherapy, relatively few (30%) have a complete response. More aggressive chemotherapy regimens are currently under investigation and may yield higher complete response rates.

An inexorable pattern of progression is characteristic, with a median time to treatment failure of less than 18 months. Median survival usually ranges from 2-5 years, and only 5-10% of patients survive 10 years.

Stage I or II localized MCL is an extremely rare presentation, and literature on its management is retrospective and anecdotal. For stage II (bulky) and stages III-IV MCL, National Comprehensive Cancer Network (NCCN) guidelines recommend induction therapy with any of several regimens; patients who show a complete or partial response should then be considered for high-dose therapy followed by autologous stem cell rescue. [8]

A consensus statement by the Lymphoma Working Party of the European Society for Blood and Marrow Transplantation and the European MCL Network (EBMT/EMCL) on the role of stem cell transplantation  in the management of MCL supported autologous SCT as the standard first-line consolidation therapy, and that complete or partial remission should be achieved before autologous SCT is performed. The EBMT/EMCL supported considering allogeneic SCT for patients who relapse after autologous SCT. [9]

Vaughn et al reported that allogeneic hematopoietic cell transplantation (HCT) provides a long-term survival benefit for patients with relapsed MCL, including those with refractory disease or multiple relapses. In this study, patients underwent HCT after nonmyeloablative conditioning with 2 Gy of total body irradiation with or without fludarabine and/or rituximab.The 5-year rates of overall survival (OS) and progression-free survival (PFS) in 70 patients were 55% and 46%, respectively. The 10-year rates of OS and PFS in 33 patients were 44% and 41%, respectively. [10]

Surgery is rarely indicated for therapeutic purposes in MCL. Exceptions include palliative procedures, such as relief of GI obstruction.


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