Mantle cell lymphomaMantle cell lymphoma is an aggressive variety of lymphoma which is typically found in the lymph nodes, spleen, marrow and blood. It is unique in that it is classified as an aggressive variety of lymphoma and yet it sometimes behaves as an indolent lymphoma. Most typically it behaves aggressively and is more difficult to treat than other indolent lymphomas.
Mantle cell lymphoma is called that because it usually infiltrates the Mantle Zone of the lymph nodes. That is the area surrounding the lymphoid follicles. When it remains in the mantle zone it generally follows a more indolent course. When it spreads outside this area, in a more diffuse growth pattern, its behaviour becomes less indolent and more aggressive.
Diagnosis and PrognosisIt is typically characterized by the expression of CD5, over expression of Cyclin D1 and the t(11:14) chromosomal translocation.
Historically mantle cell lymphoma has had a much poorer prognosis than other types of lymphoma, with a median survival of only 3-5 years. Newer chemotherapy protocols, and stem cell transplants may offer a chance for improved survival but it is too early to say with any certainty. In the era of Rituximab the media survival has improved significantly and many studies report median survival of over 7 years.
Recent discoveries have shown that the most important prognostic factor is the proliferation index as measured by the MIB-1 and Ki-67 expression. Those patients with a proliferation index below 30% have a dramatically higher survival rate. After 5 years disease free, relapses in this population group are rare and a very high percentage of patients are in this group. Those with a proliferation index above 30% generally have a much poorer prognosis. The link below will take you to a virtual education video that reviews the prognosis, and treatment of mantle cell lymphoma, the role of the proliferation index, and the prospect of a cure. Mantle Cell Lymphoma, is there a potential for a cure; from Clinical Options
Here is a somewhat technical review of the diagnosis and bio-pathology of mantle cell lymphoma from Haematologicia. Biology and bio-pathology of mantle cell lymphoma
TreatmentsMantle cell lymphoma is generally treated as an aggressive type of NHL and aggressive combination chemotherapy is used. Nevertheless in some selected cases it presents as an indolent lymphoma and watch and wait may be appropriate in these cases. R-CHOP is a standard course of treatment, but there is some evidence to show that R HyperCVAD may offer improved response and survival rates. Autologous stem cell transplants also appear to provide a superior disease free survival than R-CHOP. Below is a study which indicates that both R HyperCVAD and autologous SCT provide a superior outcome to R-CHOP.
The following MS PowerPoint slide set helps sort out all the various treatment approaches and put them into perspective. You need to create a free account with Clinical Care Options to download them, but it is well worth the effort. The series is presented by Dr. Owen A. O'Conner who is Professor of Medicine and Pharmacology Deputy Director for Clinical Research and Cancer Treatment NYU Cancer Institute Multidisciplinary Approaches for the Diagnosis and Optimal Treatment of MCL
The following is a single page chart reviewing the opinions of 10 of the foremost experts in treating mantle cell lymphoma. It asks each expert to answer the 4 questions below, and their opinions are presented in a chart. Just hover over the question for each doctor to read their answer.
A recent small study found dramatically high survival rates with a different chemotherapy regimen. At 3 years the progression free survival was 78%. While it is a small study it is worth considering for someone who is newly diagnosed. Below is a link to that study.
Another recent study examines current thinking in frontline treatments for mantle cell lymphoma. They find that adding ARA-C (Cytarabine) to chemotherapy appears to add a significant progression free survival advantage. Front-line treatment of mantle cell lymphoma
Here is a recent study from the ASH 2010 convention that examines the role of ARA-C (Cytarabine) in the treatment of mantle cell lymphoma. It shows that adding ARA-C provides a significant survival benefit.
Due to the fact that Mantle cell lymphoma generally has a worse prognosis than other aggressive types of NHL, consolidation therapy is strongly indicated. Consolidation simply means that as soon as the primary therapy is completed another therapy is initiated in order to "consolidate" the response in order to attempt to achieve a complete remission that is very durable. The most common form of consolidation therapy for most of the indolent types of lymphoma is maintenance Rituxan. It has also been shown that maintenance Rituxan dramatically improves the overall survival and progression free survival for mantle cell lymphoma patients. To view the following medical paper about maintenance Rituxan from Medscape you will need a free Medscape account. Maintenance Rituxan of benefit in mantle cell lymphoma
Two other types of consolidation therapy are common. A stem cell transplant (see below) for those who are eligible, and radioimmunotherapy such as Zevalin or Bexxar. Read the study below which shows the dramatic improvement in response when consolidation therapy is used.
Stem cell transplants are often used, either for as consolidation therapy as noted above, or for relapsed MCL. This is becoming more common as results from studies show it offers a good opportunity for long term survival. Here is a link to a recent study about using an SCT with in-vivo Rituxan purging. With a 70% overall survival at 6 years it is a remarkable improvement over past options.
Another treatment that is showing great result is Bortezomib (brand name Velcade) in many studies.
Here is an article from Annals of Oncology that reviews the current status of MCL treatments. Current treatment standards and future strategies in mantle cell lymphoma
Here is a link to a webcast (approx. 45 minutes long) from John P. Leonard, Professor of medicine, Weill Cornell Medical College. How I treat Mantle Cell Lymphoma
The following article is from the Blood Journal. It describes the current status of MCL biology and treatments. How I treat MCL. Drs. Michele Ghielmini and Emanuele Zucca
Click here for a list of medical abstracts about the latest treatments for Mantle Cell Lymphoma
Other informationFor a detailed look at Mantle Cell Lymphoma visit this site. http://www.emedicine.com/med/topic1361.htm
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