Mantle-Cell Lymphoma (Lymphoma, Mantle Cell)

A form of non-Hodgkin lymphoma having a usually diffuse pattern with both small and medium lymphocytes and small cleaved cells. It accounts for about 5% of adult non-Hodgkin lymphomas in the United States and Europe. The majority of mantle-cell lymphomas are associated with a t(11;14) translocation resulting in overexpression of the CYCLIN D1 gene (GENES, BCL-1).

Also Known As:

Lymphoma, Mantle Cell; Lymphoma, Mantle-Cell; Diffuse Lymphocytic Lymphoma, Poorly-Differentiated; Lymphocytic Lymphoma, Diffuse, Poorly Differentiated; Centrocytic Small-Cell Lymphoma; Centrocytic Small-Cell Lymphomas; Diffuse Lymphocytic Lymphoma, Poorly Differentiated; Lymphoma, Centrocytic Small Cell; Lymphoma, Mantle-Zone; Lymphomas, Centrocytic Small-Cell; Lymphomas, Mantle-Cell; Lymphomas, Mantle-Zone; Mantle Cell Lymphoma; Mantle Zone Lymphoma; Mantle-Cell Lymphomas; Mantle-Zone Lymphomas; Small-Cell Lymphoma, Centrocytic; Small-Cell Lymphomas, Centrocytic; Lymphocytic Lymphoma, Diffuse, Poorly-Differentiated; Lymphoma, Centrocytic Small-Cell; Lymphoma, Lymphocytic, Diffuse, Intermediate Differentiated; Lymphoma, Lymphocytic, Diffuse, Poorly-Differentiated; Lymphoma, Lymphocytic, Intermediate; Lymphoma, Small-Cell, Centrocytic; Mantle-Zone Lymphoma

Networked: 2988 relevant articles (265 outcomes, 469 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Neoplasms (Cancer)
2.	Non-Hodgkin Lymphoma (Lymphosarcoma)
3.	Lymphoma (Lymphomas)
4.	Multiple Myeloma
5.	Follicular Lymphoma (Lymphoma, Small Cleaved Cell, Follicular)

Experts

1.	Dreyling, Martin: 83 articles (10/2022 - 01/2003)
2.	Wang, Michael: 78 articles (11/2022 - 02/2005)
3.	Rule, Simon: 42 articles (01/2021 - 10/2010)
4.	Campo, Elias: 40 articles (12/2021 - 01/2002)
5.	Medeiros, L Jeffrey: 37 articles (10/2022 - 05/2002)
6.	Kahl, Brad S: 36 articles (05/2022 - 10/2005)
7.	Martin, Peter: 35 articles (06/2022 - 03/2008)
8.	Goy, Andre: 33 articles (06/2022 - 06/2004)
9.	Jerkeman, Mats: 33 articles (04/2022 - 01/2008)
10.	Rosenwald, Andreas: 32 articles (02/2021 - 11/2005)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Mantle-Cell Lymphoma:

1.	Rituximab (Mabthera)FDA Link 05/01/2005 - "Complete and durable remission of refractory mantle cell lymphoma with repeated rituximab monotherapy." 11/01/2018 - "Bulky primary tibia mantle cell lymphoma achieved complete remission with CHOP and DHAP plus rituximab: A case report." 09/24/2015 - "Rituximab maintenance results in improved progression-free survival but has not yet shown to improve overall survival in mantle cell lymphoma (MCL), but given the poor prognosis with post auto-HCT failure in MCL, maintenance rituximab can be considered on a case-by-case basis. " 12/01/2008 - "Although conflicting results are available, rituximab is widely used for the treatment of mantle cell lymphoma. " 09/01/2021 - "Our results confirm the long-term efficacy of autologous HSCT to treat mantle cell lymphoma established in the pre-rituximab era. "
2.	ibrutinibIBA 11/01/2019 - "Ibrutinib is highly effective in patients with relapsed or refractory mantle cell lymphoma (MCL) in major clinical trials. " 06/01/2015 - "Although ibrutinib is highly effective in patients with relapsed/refractory mantle cell lymphoma (MCL), a substantial proportion of patients have resistant disease. " 10/01/2019 - "Complete remission with ibrutinib after allogeneic stem cell transplant for central nervous system relapse of mantle cell lymphoma: A case report and literature review." 12/01/2019 - "Moreover, targeting UAE was effective in ibrutinib-resistant mantle cell lymphoma cell lines and primary cells in vitro. " 08/01/2022 - "Targeting oxidative phosphorylation or CD52 in combination with or as follow-up to ibrutinib might overcome resistance and provide improved prognosis for mantle cell lymphoma patients. "
3.	Bortezomib (Velcade)FDA Link 11/01/2015 - "Although most of the mantle cell lymphoma (MCL) patients initially responded well to bortezomib (BTZ), the dose-dependent toxicities have greatly limited the application of BTZ to MCL. " 11/10/2015 - "Inhibition of Lyn is a promising treatment for mantle cell lymphoma with bortezomib resistance." 02/01/2005 - "Encouraging activity has been demonstrated with bortezomib in the first-line treatment of myeloma and in patients with mantle cell lymphoma. " 01/01/2019 - "Association between bortezomib dose intensity and overall survival in mantle cell lymphoma patients on frontline VR-CAP in the phase 3 LYM-3002 study." 04/01/2009 - "The recent approval of bortezomib for the treatment of mantle cell lymphoma (MCL) by the US Food and Drug Administration is based on the results of the multicentre PINNACLE study with supportive data from a number of single and multicentre Phase 2 studies. "
4.	Bendamustine Hydrochloride FDA Link 12/01/2015 - "This multicentre phase II trial evaluated the pharmacokinetics (PK), safety and efficacy of bendamustine monotherapy in Chinese patients in Taiwan with pretreated indolent B-cell NHL or mantle cell lymphoma (MCL). " 01/01/2022 - "Survival in mantle cell lymphoma after frontline treatment with R-bendamustine, R-CHOP and the Nordic MCL2 regimen - a real world study on patients diagnosed in Sweden 2007-2017." 10/22/2019 - "Five-year outcomes of the S1106 study of R-hyper-CVAD vs R-bendamustine in transplant-eligible patients with mantle cell lymphoma." 07/01/2015 - "This study was a postapproval commitment to investigate bendamustine's effect on cardiac repolarization in treatment-naïve adults with advanced indolent NHL/mantle cell lymphoma (MCL). " 09/01/2010 - "In this multicenter phase II trial, the efficacy and safety of bendamustine were evaluated in Japanese patients with relapsed or refractory indolent B-NHL or mantle-cell lymphoma (MCL). "
5.	Lenalidomide (CC 5013)FDA Link 09/01/2016 - "We here present the case of refractory mantle cell lymphoma of the elderly that achieved complete remission following the use of single-agent lenalidomide, and a second complete response to the same regimen on relapse 5 years later. " 09/01/2009 - "Lenalidomide, as a single agent, induces complete remission in a refractory mantle cell lymphoma." 01/01/2018 - "In the mantle cell lymphoma (MCL)-002 study, lenalidomide demonstrated significantly improved median progression-free survival (PFS) compared with investigator's choice (IC) in patients with relapsed/refractory MCL. " 01/01/2021 - "Despite non-negligibile toxicity, lenalidomide after autologous HSCT improved progression-free survival in patients with mantle cell lymphoma, highlighting the role of maintenance in mantle cell lymphoma. " 01/01/2018 - "Lenalidomide in Pretreated Mantle Cell Lymphoma Patients: An Italian Observational Multicenter Retrospective Study in Daily Clinical Practice (the Lenamant Study)."
6.	Cytarabine (Cytosar-U)FDA LinkGeneric 02/01/2018 - "Alternating R-CHOP and R-cytarabine is a safe and effective regimen for transplant-ineligible patients with a newly diagnosed mantle cell lymphoma." 01/01/2016 - "Total body irradiation after high-dose cytarabine in mantle cell lymphoma: a comparison of Nordic MCL2, HOVON-45, and European MCL Younger trials." 01/01/2023 - "Commonly used first-line (1L) treatments for mantle cell lymphoma include high-dose cytarabine-based induction followed by autologous stem-cell transplant (ASCT) for younger patients and several chemoimmunotherapy regimens for older patients. " 09/01/2021 - "The suggested reduced efficacy after immunochemotherapy supports the need for its re-evaluation now that antibody maintenance, high-dose cytarabine, and targeted treatments have changed the standard of care for patients with mantle cell lymphoma. " 04/01/2019 - "Because of the significant activity of cytarabine in mantle cell lymphoma, increasingly, high- and intermediate-dose cytarabine are being used in the treatment of elderly mantle cell lymphoma patients. "
7.	Agammaglobulinaemia Tyrosine KinaseIBA 01/01/2019 - "Bruton's tyrosine kinase (BTK) is a key mediator of BCR-dependent cell growth signaling and a clinically effective therapeutic target in mantle cell lymphoma (MCL). " 01/01/2023 - "Commentary on: Hess et al. Real-world experience among patients with relapsed/refractory mantle cell lymphoma after Bruton tyrosine kinase inhibitor failure in Europe: The SCHOLAR-2 retrospective chart review study. " 01/01/2023 - "Real-world experience among patients with relapsed/refractory mantle cell lymphoma after Bruton tyrosine kinase inhibitor failure in Europe: The SCHOLAR-2 retrospective chart review study." 11/02/2023 - "Bruton's tyrosine kinase inhibitors (BTKi) and CAR T-cell therapy have demonstrated tremendous clinical benefits in mantle cell lymphoma (MCL) patients, but intrinsic or acquired resistance inevitably develops. " 11/01/2023 - "Bruton's tyrosine kinase (BTK) inhibitors continue to evolve in the management of mantle cell lymphoma (MCL), in both the relapsed/refractory and the frontline setting. "
8.	Proteasome InhibitorsIBA 01/01/2006 - "Mechanistic rationale and clinical evidence for the efficacy of proteasome inhibitors against indolent and mantle cell lymphomas." 12/01/2021 - "Autophagic degradation of NOXA underlies stromal cell-mediated resistance to proteasome inhibitors in mantle cell lymphoma." 06/01/2012 - "Proteasome inhibitors in mantle cell lymphoma." 09/20/2022 - "This led to the development and approval of first- and second-generation proteasome inhibitors (PIs), which have improved outcomes for patients with multiple myeloma and mantle cell lymphoma. " 01/01/2020 - "Resistance to the Proteasome Inhibitors: Lessons from Multiple Myeloma and Mantle Cell Lymphoma."
9.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 03/01/2015 - "We present a case of a 68-year-old woman who developed mantle cell lymphoma in the setting of long-term cyclophosphamide therapy and relapsing granulomatosis with polyangiitis (Wegener's granulomatosis, GPA). " 03/01/2015 - "Treatment associated Mantle Cell Lymphoma with Cyclophosphamide therapy for Granulomatosis with Polyangiitis." 05/01/2002 - "High-dose treatment with (131)I-Tositumomab, etoposide, and cyclophosphamide results in a high remission rate and may provide long-term disease-free survival for patients with relapsed or refractory mantle cell lymphoma." 11/01/2011 - "High-dose cyclophosphamide and rituximab without stem cell transplant: a feasibility study for low grade B-cell, transformed and mantle cell lymphomas." 09/01/2001 - "Cyclophosphamide/fludarabine (CF) is active in the treatment of mantle cell lymphoma."
10.	venetoclaxIBA 01/01/2020 - "Efficacy of venetoclax in high risk relapsed mantle cell lymphoma (MCL) - outcomes and mutation profile from venetoclax resistant MCL patients." 01/01/2020 - "Venetoclax is effective in relapsed patients with mantle cell lymphoma (MCL). " 11/01/2023 - "In a multivariable analysis, having a high-risk Mantle Cell Lymphoma International Prognostic Index score before receiving venetoclax and disease relapse or progression within 24 months of diagnosis were associated with inferior OS whereas the use of venetoclax in combination was associated with superior OS. " 11/01/2023 - "A multicenter analysis of the outcomes with venetoclax in patients with relapsed mantle cell lymphoma." 01/13/2023 - "Concurrent Inhibition of Akt and ERK Using TIC-10 Can Overcome Venetoclax Resistance in Mantle Cell Lymphoma."

Therapies and Procedures

1.	Therapeutics 01/01/2020 - "Potent efficacy of MCL-1 inhibitor-based therapies in preclinical models of mantle cell lymphoma." 05/01/2015 - "Although there is a lack of consensus regarding the optimal management strategy of mantle cell lymphoma, novel targeted therapies show promise in extended remission." 08/31/2023 - "Chemoimmunotherapy followed by consolidative high-dose therapy with autologous stem cell rescue was a standard upfront treatment for fit patients with mantle cell lymphoma (MCL) in first remission; however, treatment paradigms are evolving in the era of novel therapies. " 12/01/2018 - "Novel targeted therapeutics has significantly improved the outlook of patients with relapsed/refractory mantle cell lymphoma (R/R MCL). " 05/01/2002 - "[Primary subcutaneous mantle cell lymphoma treated successfully with THP-COP therapy]."
2.	Drug Therapy (Chemotherapy) 01/01/2019 - "Low-dose radiation (4 Gy) with/without concurrent chemotherapy is highly effective for relapsed, refractory mantle cell lymphoma." 01/01/1998 - "In advanced stage mantle cell lymphoma, conventional chemotherapy yields a complete remission rate below 40%, and the median survival rate is only about 3 years. " 05/02/2007 - "In patients with indolent or mantle cell lymphoma, R-chemo is superior to chemotherapy alone with respect to overall survival." 01/01/2019 - "Our previous study demonstrated that knocking down of B7H3 inhibited cell proliferation, invasion and enhanced the therapeutic efficacy of chemotherapy in mantle cell lymphoma (MCL). " 04/15/2021 - "Renal impairment was improved by chemotherapy for the mantle cell lymphoma. "
3.	Stem Cell Transplantation 12/15/2016 - "Counterpoint: The Role of Stem Cell Transplantation in Mantle Cell Lymphoma: The Pendulum Swings Again: Time to Reassess the Role of ASCT in First Complete Remission." 09/01/2016 - "PET-CT confirmed complete remission and MRD negativity in mantle cell lymphoma patients treated with R-hyper-CVAD results in excellent outcome in the absence of autologous stem cell transplantation: a single-centre case for a transplant-free approach." 02/01/2000 - "This study was designed to evaluate the efficacy of therapeutic intensification with autologous stem cell transplantation (ASCT) for mantle cell lymphomas (MCL) in terms of response rate, duration of response, and event-free and overall survivals. " 02/25/2010 - "The Mantle Cell Lymphoma International Prognostic Index (MIPI) is superior to the International Prognostic Index (IPI) in predicting survival following intensive first-line immunochemotherapy and autologous stem cell transplantation (ASCT)." 08/01/2009 - "Autologous and allogeneic stem cell transplantation: rising therapeutic promise for mantle cell lymphoma."
4.	Transplantation 01/01/2005 - "The benefit of autologous or allogeneic BMT for mantle cell lymphoma is thus most apparent when transplantation is performed in first remission. " 11/01/2001 - "Optimal first-line regimens for mantle cell lymphoma have yet to be defined, and it is therefore difficult to place the role of first remission transplantation in an appropriate context. " 08/01/1996 - "Clinical and molecular remission after allogeneic blood cell transplantation in a patient with mantle-cell lymphoma." 04/30/2009 - "In this study, we analyzed the long-term outcome of a risk-adapted transplantation strategy for mantle cell lymphoma in 121 patients enrolled in sequential transplantation protocols. " 02/01/1999 - "The results of transplantation trials for these patients are also confounded by the relatively short follow-up intervals in low-grade NHL and small patient numbers in studies of transplantation for less common NHL subtypes, such as lymphoblastic, Burkitt's, and mantle cell lymphomas. "
5.	Autologous Transplantation 02/01/2001 - "Central nervous system relapse in a patient with mantle cell lymphoma in continuous clinical and molecular remission at six years since autografting." 03/01/2003 - "Outcome of autologous transplantation for mantle cell lymphoma: a study by the European Blood and Bone Marrow Transplant and Autologous Blood and Marrow Transplant Registries." 09/01/2012 - "Autotransplantation for mantle cell lymphoma." 06/01/2012 - "Autologous transplantation and management of younger patients with mantle cell lymphoma." 10/01/2008 - "Long-term progression-free survival after early autologous transplantation for mantle-cell lymphoma."