Mantle cell lymphoma (MCL) is a B-cell lymphoma that has previously been called diffuse small-cleaved cell lymphoma. Sometimes it was called intermediate differentiation lymphoma or centrocytic lymphoma. Despite all these names, it is not common (2.5% to 4% of all Nonhodgkin's lymphomas in the US and 7% to 9% of lymphomas in Europe).
Presentation
MCL primarily afflicts men over 50, who almost always present with advanced, Stage III or IV disease. Bone marrow involvement is seen in 60% to 90% pf patients. Four histologic subtypes have been noted - Nodular, Diffuse, Mantle Zone and Blastic.
Blastic appears to have the worst prognosis with Nodular and Diffuse having longer average survival times.
Treatment
Mantle cell lymphoma is an aggressive non-Hodgkin's lymphoma that historically been resistant to current standard chemotherapeutic approaches.
Despite response rates to many regimens of 50% to 70%, the disease would progress after chemotherapy with a median survival time of approximately 2.5 to 4 years.
In 2005, there are some newer treatments but there appears to be no clear standard approach for treating mantle cell lymphoma, making it critical that appropriate patients be enrolled in clinical trials.
High-dose therapy with autologous
stem cell transplantation may provide longer time to progression. For young patients with matched donors, allogeneic transplant is promising in the limited numbers of patients treated.
Immunotherapy treatments under development may be the best answer to mantle cell treatment.
Rituxan in combination with
CHOP (CHOP-R) has a 96% response rate. Use of this treatment provides a prolonged response compared to chemotherapy alone but studies show many patients still relapse. At Stanford University Medical Center, they have a protocol involving treatment with CHOP-R followed by a BMT using VP-16 and total body irradiation. Very intense but their lasting remission rate is very high.
As mantle cells may remain after treatment, researchers are looking at use of rituxan as a maintenance therapy (see references).
In the US, one study is looking at a drug called
VELCADE™ (bortezomib). Another promising therapy in clinical trials is
Genasense ™ - a BCL-2 protein blocker.
Finally teams including MD Anderson Cancer Center are looking at vaccine therapy for Mantle Cell Lymphoma. (see references)
Ask your medical professional about treatment options now available.
Resources
Information
Good Paper by eMedicine
A good writeup from Dr. Kevin Imrie
The Mantle Cell Lymphoma page on Lymphoma and Integrative Medicine is comprehensive
A paper on Mantle Cell by UCSF
Information from Hematopathology
1999 Paper American Society of Hematology - Mantle Cell
Information from a pathology update in the Cancer Control Journal April 1996: Indolent Lymphomas
Lymphomation.org - Mantle Cell Lymphoma
Cancerbackup (UK): Mantle cell lymphoma
Mantle Cell Lymphoma Site by Ron Edwards
Treatment
BMT Information (excellent)
ABMT in Patients with Low Grade or Mantle Cell Lymphoma (2001)
Lymphoma: New Treatment Possibilities
ASBMT Paper 2000 Vose, JM et al. Autologous HSCT for Mantle Cell Lymphoma
Mailing Lists
See the mailing list page for NHL (many) and Mantle Cell (1) mailing lists
References
Hiddemann W, Forstpointer R, Dreyling M, et al. Rituximab maintenance following a rituximab containing chemotherapy significantly prolongs the duration of response in patients with relapsed follicular and mantle cell lymphoma: Results of a prospective randomized trial of the German Low Grade Lymphoma Group (GLSG). Proceedings from the 41st annual meeting of the American Society of Clinical Oncology. Orlando FL. 2005; Abstract #6527.
Forstpointer R, Dreyling M, Repp R, et al. The addition of rituximab to a combination of fludarabine, cyclophosphamide, mitoxantrone (FCM) significantly increases the response rate and prolongs survival as compared with FCM alone in patients with relapsed and refractory follicular and mantle cell lymphomas: results of a prospective randomized study of the German Low-Grade Lymphoma Study Group. Blood . 2004;104:3064-3071.
Neelapu SS, Kwak LW, Kobrin CB, Reynolds CW et al., Vaccine-induced tumor-specific immunity despite severe B-cell depletion in mantle cell lymphoma, Nature Medicine, 2005 Sep;11(9):986-91. Epub 2005 Aug 21
Abstract
Histopathology
MCL has pan-B cell antigens CD19, CD20, and CD22 with surface IgM and IgD in addition to the pan-T antigen CD5. CD5 distinguishes MCL from
follicular lymphomas which rarely express this antigen. The lack of CD23 and a higher antigen density of CD20 and surface Ig is useful in distinguishing MCL from
small lymphocytic lymphoma. MCL has a unique cytogenetic translocation involving chromosomes 11 and 14 t(11;14)(q13;q32). This juxtaposes the enhancer element of the Ig heavy chain JH region on chromosome 14 to the bcl-1 proto-oncogene which encodes the cyclin D1 protein on chromosome 11. The overexpression of cyclin D1 produces excessive B-Cell proliferation - this is seen in 90% of MCL cases and is considered a reliable diagnostic marker.
Information Sources
The Hematopathology of Lymphoma - technical but full of very good information.
NCI Non-Hodgkin's Lymphoma Information
The Atlas of Genetics and Cytogenetics in Oncology and Haematology page on MCL
Frontiers in Bioscience is a source of B-cell information.
Unusual Lymphoma Entities: Evaluation and Management by Vose, Fisher, and Lister, 1997 ASCO
Abstract: Mantle Cell Lymphoma, Campo E. et al., Semin Hematol. 1999 Apr;36(2):115-27
Abstract: Mantle Cell Lymphoma, Densmore JJ, Williams ME, Curr Treat Options Oncol. 2003 Aug;4(4):281-7
Mantle Cell Lymphoma - A Difficult to Treat Lymphoma: Present Therapy and New Developments, Greer, John MD, Lymphoma Today (Lymphoma Research Foundation Newsletter) vol 2 no 4 Winter 2003
Related Articles
Non-Hodgkin's Lymphoma Information Pages:
Non-Hodgkin's Lymphomas
Non-Hodgkin's Lymphoma: Diagnosis
Non-Hodgkin's Lymphoma: Aggressive Lymphomas
Non-Hodgkin's Lymphoma: Indolent Lymphomas
Non-Hodgkin's Lymphoma: Treatment
Non-Hodgkin's Lymphoma: Resources
Bone Marrow and Stem Cell Transplants
Other Pages of Interest:
Childhood Non-Hodgkin's Lymphoma Information
The Lymphoma Information Network
Books
Non-Hodgkin's Lymphomas, Peter M. Mauch (Editor), James O. Armitage (Editor), et al., 2004. Technical but very good
Section IV: Pathology, Biology, Clinical Evaluation, and Treatment Section
Chapter 29: Mantle Cell Lymphoma
The Patient from Hell: How I Worked with My Doctors to Get the Best of Modern Medicine and How You Can Too by Stephen H. Schneider, Ph.D., Da Capo Press, Cambridge, MA, 2005 0-7382-1025-0 - a must for any Mantle Cell Lymphoma patient or loved one.
New, Excellent but Technical:
An Illustrated Guide to Skin Lymphomas, Cerroni, Gatter, Kerl, 2nd. Ed, 2005 pp 126-7
