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 Follicular Lymphoma  

Follicular lymphoma 
This is the most common of the indolent lymphomas and accounts for about 70% of them, and about 22% of all lymphomas in North America and Europe. At the bottom of this page is an explanation of where the name follicular comes from and what it looks like under the microscope.

Follicular lymphoma is divided into 3 grades. Grade 1, 2 or 3. However Grade 3 is somewhat blurry because it can appear to the the indolent form of follicular lymphoma or it can appear to be the more aggressive form. The grade refers to the number of large cells that appear under the microscope. Large cells tend to behave a bit more aggressively than small cells. Although more large cells appear in Grade 2, it is for all intents and purposes considered the same as Grade 1 from a prognosis and treatment point of view. If you are confused about the difference between grade and stage you are not alone. Here are links to the two definitions.

Grade explanation

Stage explanation

Follicular lymphoma is a slow growing type of NHL which responds very well to treatment, but can rarely be cured. Despite the fact that it cannot be cured, the prognosis is generally very good and the median survival is about 8-15 years according to the NCI. However one recently published study from Stanford University school of medicine has shown that since 1997 the median survival has increased to over 18 years.
Read the Stanford abstract here.

Despite the fact that fNHL is considered incurable it can usually be managed very well and it responds well to treatment. Some even consider it a chronic disease rather than a terminal one. The choice of treatment depends on many factors, but usually a watch and wait approach is appropriate for patients who are not exhibiting any symptoms, who do not have bulky disease, or who do not have any major organs threatened by their tumours. Some patients may be uncomfortable with W&W and prefer to take some active action against their disease. However studies have consistently shown that the survival is the same whether treatment is initiated immediately or deferred until needed. Similarly, treating aggressively upfront does not improve the survival over treating with minimal necessary treatment. Patient and doctor preferences must both be considered when choosing treatment.

When treatment is initiated there are many options to choose from. There are no right or wrong choices and patient preference often plays a role. Some prefer the mildest effective treatment to preserve lifestyle and healthy living, others prefer more aggressive upfront treatment for peace of mind and the feeling of eradicating the disease. Both approaches have merit. Listed here are options from mildest to more aggressive.

Oral Chlorambucil is a very effective treatment though it has fallen out of favour in recent years. Lack of profit for the prescription drug is a motivating factor. Nevertheless this alkyating agent is highly effective in fNHL, very convenient, and has an excellent side effect profile.

Single agent Rituxan is another choice. Rituxan is a monoclonal antibody therapy and not chemotherapy. You can read more about monoclonal antibodies by clicking here.  Although single agent Rituxan has only moderate efficacy it is very mild in terms of treatment and does not burn any future treatment options. It can be an excellent choice for those who need treatment but are wishing for minimal intervention. Where it really shines though is when combined with chemotherapy. In that setting there appears to be a highly synergistic effect.

Next would be combination chemotherapy such as:

CVP+Rituxan (R-CVP)
CHOP + Rituxan (R-CHOP)
Radioimmunotherapy such as Bexxar or Zevalin
Fludarabine + Rituxan (RF)
Fludarabine combination such as FND or FC; both with Rituxan
Vaccine therapies are in clinical trials as well

Click here to see a complete list of common chemotherapy protocols for NHL

What about Stem Cell Transplants? Should they be used for follicular lymphoma? Here is some additional information on this very controversial topic from the Journal of Clinical Oncology. It is a very provocative discussion.

Should We Transplant Indolent Lymphoma?
http://www.jco.org/cgi/content/full/23/25/6263-a

The reply from the author of the original study (see below)
http://www.jco.org/cgi/content/full/23/25/6264

The original study
Moderate Increase of Secondary Hematologic Malignancies After Myeloablative Radiochemotherapy and Autologous Stem-Cell Transplantation in Patients With Indolent Lymphoma: Results of a Prospective Randomized Trial of the German Low Grade Lymphoma Study Group
 

There are also vaccine therapies in the pipeline, some of which are very near to FDA approval in the USA. Vaccines use various methods of taking the patients own tumour cells, and then producing a vaccine that is unique to that patient.  Some studies are showing dramatically increased remission times using vaccines. For more information about vaccine therapies you should start by going to the websites of the three companies that are currently doing clinical trials in vaccine therapy, and that are close to marketing approvals.

Biovest International BiovaxID

Genitope Corporations MyVax

Favrille Inc. FavID

Click here for a more complete list of drugs in the pipeline for non-hodgkin's lymphoma

So why is fNHL considered a chronic disease? Here are a couple links to studies that shows how survival for fNHL has improved in recent decades.

New Treatment Options Have Changed the Survival of Patients With Follicular Lymphoma

Improvement of Overall and Failure-Free Survival in Stage IV Follicular Lymphoma: 25 Years of Treatment Experience

 

Here are some links to additional information about follicular lymphoma

E-medicine follicular lymphoma

NCI information about indolent lymphomas

 


The tumour microenvironment is currently a very hot area of research. This is referring to the role the healthy cells in the tumour behaviour and prognosis of follicular NHL.

Click here for more detailed information about the microenvironment


Where the name "follicular" comes from.

The name of this lymphoma derives from the location and behaviour of the cancerous cells. They usually originate in the lymphoid follicles, but more important is that they grow in a follicular pattern. This means that the cells tend to clump together or "stick" together. 

normal.jpg (88200 bytes)

Above is an image of a normal lymph node slice at a medium magnification. Notice how it has those round "follicles" around the outer top edge. They are the pale purple circles you see.  That is what a normal lymph node should have. For a drawing of the same thing click here:

follicular.jpg (18641 bytes)

Here is slide of a lymph node with follicular lymphoma. Notice how many more of those follicles there are! And notice how tightly packed they are throughout the node, rather than just at the outer edge.

While follicular lymphomas very often form outside of the lymph nodes, it is this follicular pattern of growth that gives it its name. (past classification systems called it nodular) 

 

 

 

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