Mantle cell lymphoma
   

  

Mantle cell lymphoma

Mantle 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 Prognosis

It 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.

 

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

 

 

Treatments

Mantle 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.

Effect of front-line therapy with either high-dose therapy and autologous stem cell rescue (HDT/ASCR) or dose-intensive therapy (R-Hypercvad) on outcome in mantle cell lymphoma (MCL)

 

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.

High rate and prolonged duration of complete remissions induced by rituximab, methotrexate, doxorubicin, cyclophosphamide, vincristine, ifosfamide, etoposide, cytarabine, and thalidomide (R-MACLO-IVAM-T), a modification of the National Cancer Institute 89-C-41 regimen, in patients with newly diagnosed mantle cell lymphoma

 

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

 

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.

 

Two 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.

 

Survival benefit of post induction consolidation therapy in MCL (mantle cell lymphoma): A Polish Lymphoma Research Group (PLRG) retrospective multicenter analysis.

 

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.

 

Long-term progression-free survival of mantle cell lymphoma after intensive front-line immuno-chemotherapy with in vivo–purged stem cell rescue

  

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 information

For a detailed look at Mantle Cell Lymphoma visit this site.

http://www.emedicine.com/med/topic1361.htm