Follicular lymphomaThis 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. See more information below about grade 3 follicular. If you are confused about the difference between grade and stage you are not alone. Here are links to the two definitions.
Diagnosis and PrognosisFollicular 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.
One of the primary concerns for any patient with follicular or other indolent lymphoma is transformation to a more aggressive lymphoma such as diffuse large B-cell lymphoma. Click the link below to read more about transformation. Information about Transformation
Below is a detailed description of the pathology of follicular lymphoma. It may help to give a better understanding of exactly how it occurs and some of the challenges understanding it.
Molecular pathogenesis of follicular lymphoma
This type of follicular lymphoma has commonly been associated with a worse overall survival rate. But this is highly controversial, and different studies show different results. The follow study reveals that dividing grade 3 into 3a (similar to grade 1 & 2) versus grade 3b (similar to diffuse large B-cell) is not exact enough. It must be divided into grade 3 with less than 50% diffuse component, versus grade 3 having more than 50% diffuse component. Grade 3 with <50% has an outcome very similar to grade 1 & 2.
Here is more information about low grade and grade 3 fNHL
TreatmentsIn many cases treatment may be deferred and a watch and wait approach is appropriate. Studies have consistently shown that there is no survival benefit to treating immediately versus waiting until treatment is necessary. Some of the factors that may indicate the need for treatment include:
That last reason is a common one. There are many people who just cannot wrap their head around the concept of having cancer and doing nothing about it. This is a valid option for those who so desire.
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)
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?
The reply from the author of the original study (see below) http://www.jco.org/cgi/content/full/23/25/6264
The original study
Here is a study that looks at the long term outlook for people who have had a relapse after an SCT. It also reports on the risk of relapse after SCT (less than 50% relapse rate.
Other informationIs follicular lymphoma incurable?Perhaps this is not as true as it has been in the past. Below are some recent studies which show how long term survival is becoming more common for follicular lymphoma.
Aiming for a curative strategy for follicular lymphoma
New Treatment Options Have Changed the Survival of Patients With Follicular Lymphoma
Here is an excellent Continuing Medical Education article (CME) which discusses whether or not radioimmunotherapy offers the chance to cure follicular NHL. (Paid subscription required) Radiolabeled and Native Antibodies and the Prospect of Cure of Follicular Lymphoma
In 1998 a study was started which examined using extended Rituximab to see how it would affect survival of patients with fNHL. The first results from that study were published in Blood in 2004 with impressive results. Here is that study.
In 2009 follow up results were published at the ASCO convention. Those results confirmed the earlier results. Here is that abstract.
Medscape has an excellent article which discusses the above study and puts it into perspective and how a cure may actually be achievable. Medscape requires a membership to view its articles, but membership is free and takes only a minute to setup. ASCO 2009: Prolonged Rituximab Extends Remission in Follicular Lymphoma
The following article from Bloodline reviews reduced intensity conditioning allogeneic transplants (RIC) (aka Mini-transplants) Starting on page 7 it addresses lymphoma. The first study they review shows that for follicular lymphoma treated with RIC. At 9 years more than 80% of patients are still disease free.
Here are some links to additional information about follicular lymphoma. E-medicine follicular lymphoma
NCI information about indolent lymphomas Click here for more detailed information about the microenvironment
Where the name "follicular" comes fromThe 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.
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:
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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