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Mantle Cell Lymphoma (MCL)

  

About Lymphoma > Types of Lymphoma > Mantle Cell Lymphoma (MCL)

Last update: 05/14/2009

On this Page:  Overview | Treatments | Clinical Trials | Prognosis & Response to Treatment

TOPIC SEARCH: PubMed: Diagnosis | Review | Therapies | Prognosis
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ABOUT Lymphomas

Overview of genes and cancer

Lymphoma is a cancer

About Lymphoma - general
Characteristics of NHL
  Cell type | Histology | Grading | Staging

  Ann Arbor Staging 
  Extranodal notations  

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Treatment Decisions
  
Treatments

Watch & Wait
  

Factors that determine treatment timing and approach

The characteristics of the lymphoma at diagnosis as determined by the pathology report, and it's actual clinical behavior, and other factors determine the type of treatment and the timing of treatment you and your doctor will consider. 

See Factors that influence treatment selection and timing  PAL
 
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Mantle Cell non-Hodgkin's Lymphoma

A cancer of b-cells (lymphocytes) that normally reside in lymph nodes.   

"Mantle cell" describes the cell type. B-cells arise from the bone marrow and mature or differentiate into many cell types that tend to migrate to different areas of the body. Most lymphoma results from an acquired (not inherited) injury to the DNA in the genes of a single cell. 

A hallmark of cancer cells is that they have growth and survival advantages over normal cells. There cell division is not balanced by cell death. The abnormal cells may eventually form lumps called tumors. 

See Image of Marginal Zone b-lymphocytes

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Incidence

Mantle Cell Lymphoma represents 4% to 8% of non-Hodgkin’s lymphomas. It primarily affects older individuals; males more than females by a ratio of about 4 to 1. The median age at diagnosis is approximately 58 years of age. 

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Diagnosis   

To make an accurate diagnosis of lymphoma, a biopsy must be performed by the surgical removal (resection) of a lymph node.  A fine needle aspiration may be performed if a lymph node is not accessible, but this is not considered a definitive way to determine the diagnosis.

A series of tests will then be performed to determine the characteristics of the cells.  If a malignancy is determine, these characteristics will allow your doctors to determine the appropriate treatments to use when needed.  

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Workup   

See for details  e-medicine

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Common symptoms

fatigue (anemia)
loss of appetite
feeling of fullness or discomfort due to enlarged liver or spleen
enlarged lymph nodes - painless swelling in the neck, armpit or groin - often in more than one group

Other symptoms may include night sweats, unexplained high temperatures and weight loss. These are known as B symptoms.

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Resources
About  Emedicine | LLS
Mantle Cell Lymphoma: Background for physicians
Medscape.com (free login req.)

Mantle cell lymphoma (MCL), which represents approximately 6% of non-Hodgkin's lymphoma (NHL) cases, remains the most challenging lymphoma to treat. As a result, this is one of the most active areas of investigation in lymphoma, in terms of translational research as well as in the development of novel approaches, some of which will be highlighted in this review.
Non-Hodgkins Lymphomas - Clinical Practice Guidelines in Oncology 
nccn.org professionals pdf 
Genetics  Cancer Genetics Web 
Mantle Cell Information Websites  mclresource.com/MCLAid 
CD5- Mantle Cell Lymphoma:  from American Journal of Clinical Pathology - Medscape (free login req.) 
 
"Mantle cell lymphoma (MCL) typically expresses B-cell antigens and CD5 and overexpresses bcl-1 protein. However, unusual cases of bcl-1+ and CD5- MCL have been observed, posing a practical challenge for correct diagnosis and management." 
Mantle Cell Lymphoma: Background  Medscape.com (free login req.)

Mantle cell lymphoma (MCL), which represents approximately 6% of non-Hodgkin's lymphoma (NHL) cases, remains the most challenging lymphoma to treat. As a result, this is one of the most active areas of investigation in lymphoma, in terms of translational research as well as in the development of novel approaches, some of which will be highlighted in this review.
Treatments
Questions for your doctor
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Treatment Overview
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Treatment articles for MCL:

TOPIC SEARCH on Treatment of MCL
PubMed:  Review | Therapies | Rituxan | Stem Cell Transplant 
ASCO | Medscape | FDA | WEB  
Agents/Protocols: Velcade (Bortezomib) | HyperCVAD

Also see 
Protocols for Refractory Disease  PAL 
Clinical Trials for MCL  PAL
Limited-stage mantle-cell lymphoma
H. A. Leitch1, R. D. Gascoyne2, M. Chhanabhai2, N. J. Voss3, R. Klasa1 and J. M. Connors
http://annonc.oxfordjournals.org/cgi/content/full/14/10/1555 
Revlimid® Active for Treating Mantle Cell Lymphoma cancerconsultants.com

n = 15 patients with mantle cell lymphoma who had failed prior therapy, including stem cell transplantation in five. 

The overall response rate was 53% with 20% (n=3) having a complete response. The median duration of response was 14 months, and the median progression-free survival was 5.6 months. Four of the five patients who had failed stem cell transplantation responded.
  Phase II multicenter study of bendamustine plus Rituxan in patients with relapsed indolent B-cell and mantle cell NHL.  
J Clin Oncol. 2008 Sep 20;26(27):4473-9. Epub 2008 Jul 14. PMID: 18626004  

(n = 66 patients with relapsed, indolent B-cell or mantle cell lymphoma (without documented resistance to prior rituximab)

Overall response rate was 92% (41% complete response, 14% unconfirmed complete response, and 38% partial response). Median duration of response was 21 months (95% CI, 18 to 24 months). Median progression-free survival time was 23 months (95% CI, 20 to 26 months). Outcomes were similar for patients with indolent or mantle cell histologies.
Long-term progression-free survival of mantle cell lymphoma following intensive front-line immunochemotherapy with in vivo-purged stem cell rescue: A non-randomized phase-II multicenter study by the Nordic Lymphoma Group. Blood. 2008 Jul 14.  PMID: 18625886   | full text bloodjournal.hematologylibrary.org  

(n = 160 consecutive, untreated patients younger than 66 years)
 Managing and avoiding bortezomib (Velcade) toxicity 
Jeffrey Menashe, MD | Northwest Cancer Specialists, Portland, OR
 communityoncology.net pdf
Mantle Cell Lymphoma Can Be Cured by Intensive Immunochemotherapy with In-Vivo Purged Stem-Cell Support; Final Report of the Nordic Lymphoma Group MCL2 Study  ASH abstracts2view.com 

Conclusion: The demonstration of long-term event-free survival in a large, consecutive prospective series now for the first time indicates that intensive immunochemotherapy including AraC and Rituximab with in-vivo purged stem-cell support may cure mantle cell lymphoma.
New Zevalin in Mantle Cell Lymphoma touchbriefings.com   pdf  

Conclusion: Radiotherapy is an active treatment modality in MCL. Ibritumomab radioimmunotherapy (Zevalin) is an interesting alternative to other consolidation methods. In younger patients subjected to intensive chemotherapy followed by autologous transplant, it may be an element of a transplant conditioning regimen (i.e. Z-BEAM). In elderly patients, the role of zevalin consolidation should be further investigated in a phase III trial.
New directions in the treatment of mantle cell lymphoma: an overview.
Clin Lymphoma Myeloma. 2006 Oct;7 Suppl 1:S24-32. PMID: 17101070 | Related articles
Emerging New Strategies in the Treatment of Mantle Cell Lymphoma - Section III of asheducationbook.org/cgi/content/full/2004/1/221 
High Rate of Durable Remissions After Treatment of Newly Diagnosed Aggressive Mantle-Cell Lymphoma With Rituximab Plus Hyper-CVAD Alternating With Rituximab Plus High-Dose Methotrexate and Cytarabine.  ASCO 2005
RQ-PCR Positivity Predicts Relapse of Mantle Cell Lymphoma 
after Autologous Stem Cell Transplant  cancerconsultants.com

The researchers concluded that the presence of MRD (minimal residual disease) following an AutoSCT in patients with MCL is highly predictive of outcomes in this group of patients. The authors suggest testing for MRD in this group of patients; those with MRD may benefit from additional or more aggressive therapies.
High-Dose Iodine-131-Labeled Rituxan and Autologous Stem Cell Support Produces Long Lasting Effects in Patients with Mantle Cell Lymphoma Failing Conventional Autologous Transplants  cancerconsultants.com
Rituximab (Rituxan ®) Improves Response Rate in Mantle Cell Lymphoma  Cancer.gov  
Non-Hodgkin’s lymphoma, mantle cell lymphoma, rituximab (Rituxan®), monoclonal antibody, targeted therapy. 

(Definitions of many terms related to cancer can be found in the Cancer.gov Dictionary.)  Summary:  Rituximab, when added to standard chemotherapy for initial treatment of mantle cell lymphoma, shrank or eliminated tumors in a significantly higher percentage of patients than the standard chemotherapy (CHOP) alone. Further follow-up is needed to show whether this approach also increases overall survival time."
Phase II Trial of Single-Agent Temsirolimus (CCI-779) for Relapsed Mantle Cell Lymphoma. J Clin Oncol. 2005 Jun 27  PMID: 15983389
Mantle Cell Lymphoma: At Last, Some Hope for Successful Innovative Treatment 
Strategies ~ Editorial by Dr.  Richard I. Fisher  jco.org 
Treatment of Non-Hodgkin's Lymphoma: Next Steps  Medscape.com 2004 (free login req.) Review of progress for Follicular, SLL, Diffuse Large Cell, and Mantle Cell.
Studies presented at ASH explore activity of the Bexxar® therapeutic regimen in difficult-to-treat lymphomas  www.icaa.eu
MCL - Treatment policy prepared by Dr. Kevin Imrie  - Updated Feb 2003: minor changes on rituximab  tsrcc.on.ca - PDF
" Mini " Transplants Effective in Recurrent Mantle Cell   cancerconsultants.com
Fludarabine-based chemotherapy in untreated mantle cell lymphomas:
an encouraging experience in 29 patients PIER LUIGI ZINZANI et al. - Haematologica 1999; 84:1002-1006 PDF
A low toxicity maintenance regime, using eicosapentaenoic acid and readily available drugs, for mantle cell lymphoma and other malignancies with excess cyclin D1 levels. Med Hypotheses. 2003 May;60(5):615-23. PMID: 12710892  PubMed
Velcade: Inhibition of the proteasome induces cell cycle arrest and apoptosis in mantle cell lymphoma cells. Br J Haematol. 2003 Jul;122(2):260-8. PMID: 12846895  PubMed
The Role of Autologous Transplantation in the Management of Mantle Cell Lymphoma: A Study From the EBMT , E. Vandenberghe, et al.  PDF - mmserver.cjp.com
Review of :"Long-term remission in mantle-cell lymphoma following high-dose sequential chemotherapy and in vivo rituxan-purged stem cell autografting (R-HDS regimen)" Blood  Docguide.com  
The Role of Autologous Transplantation in the Management of Mantle Cell Lymphoma:  A Study From the EBMT E. Vandenberghe, C. Ruiz de Elvira, P. Isaacson, E. Lopez-Guillermo, E. Conde, C. Gisselbrecht, F. Guilhot, A. Goldstone, N. Schmitz 
PDF | PDF-Help 
Mantle Cell treatment effective  BBCNews  
Response to thalidomide in chemotherapy-resistant mantle cell lymphoma: a case report. Br J Haematol. 2002 Oct;119(1):128-30. PMID: 12358916  PubMed
Mantle cell lymphoma involving skin: cutaneous lesions may be the first manifestation of disease and tumors often have blastoid cytologic features.
Am J Surg Pathol. 2002 Oct;26(10):1312-8. PMID: 12360046  PubMed
Tandem Transplants: Patients with mantle-cell lymphoma relapsing after autologous stem cell transplantation may be rescued by allogeneic transplantation. Bone Marrow Transplant. 2000 Sep;26(6):677-9. PMID: 11035375   PubMed
Temsirolimus Promising for Treatment of Mantle Cell Lymphoma, and other cancers  cancerconsultants.com
Clinical Trials
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MCL prognostic factors: A new prognostic index (MIPI) for patients with advanced stage mantle cell lymphoma. Blood. 2007 Oct 25  PMID: 17962512

According to the MIPI, patients were classified into low risk (44% of patients, median OS not reached), intermediate risk (35%, 51 months), and high risk groups (21%, 29 months), based on the four independent prognostic factors: (1) age,  (2) ECOG performance status, (3) LDH and leukocyte count. 

Cell proliferation (Ki-67)  was exploratively analyzed as important biological marker and showed strong additional prognostic relevance. 
High Rate of Durable Remissions After Treatment of Newly Diagnosed Aggressive Mantle-Cell Lymphoma With Rituximab Plus Hyper-CVAD Alternating With Rituximab Plus High-Dose Methotrexate and Cytarabine.  ASCO 2005
Mantle cell lymphoma: prognostic capacity of the 
Follicular Lymphoma International Prognostic Index (FLIPI).
Br J Haematol. 2006 Apr;133(1):43-9. PMID: 16512827 | See FLIPI
Allogeneic stem cell transplantation for mantle cell lymphoma--does it deserve a better look? Leuk Lymphoma. 2005 Feb;46(2):217-23. PMID: 15621804
MCL prognostic factors:  The Leukemic Presentation of Mantle-cell Lymphoma: Disease Features and Prognostic Factors in 58 Patients. Leuk Lymphoma. 2004;45(10):2007-2015. PMID: 15370245   

"This analysis demonstrates that except for splenomegaly, survival of MCL patients presenting with leukemia is not significantly influenced by clinical or tumor characteristics. Splenectomy is a useful treatment option in this group of patients."
Allogeneic “Mini” Stem Cell Transplant Effective for Recurrent Mantle Cell Lymphoma  cancerconsultants.com
Hyper CVAD Followed by SCT for MCL  PubMed
Long-term follow-up of autologous stem cell transplantation in patients with diffuse mantle cell lymphoma in first disease remission: the prognostic value of beta2-microglobulin and the tumor score. Cancer. 2003 Dec 15;98(12):2630-5. 
PMID: 14669282 | Related articles
Survivin Expression in Mantle Cell Lymphoma I ... The role of survivin in the pathogenesis of mantle cell lymphoma (MCL) was examined in a series of typical and blastoid tumors. amjpathol.org 
Sequential chemotherapy by CHOP and DHAP regimens followed by high-dose therapy with stem cell transplantation induces a high rate of complete response and improves event-free survival in mantle cell lymphoma: a prospective study.
Leukemia. 2002 Apr;16(4):587-93.
PMID: 11960337  PubMed
Factors affecting toxicity, response and progression-free survival in relapsed patients with indolent B-cell lymphoma and mantle cell lymphoma treated with rituximab: a Japanese phase II study. Ann Oncol. 2002 Jun;13(6):928-43.
PMID: 12123339  PubMed
 
Disclaimer:  The information presented on Lymphomation.org is not intended to be a substitute for 
professional medical advice or to replace your relationship with a physician.
For all medical concerns,  you should always consult your doctor. 
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