Follicular Lymphoma: Symptoms, Diagnosis, Treatment, and Prognosis
What Is Follicular Lymphoma?
Follicular lymphoma is a slow-progression type of non-Hodgkin lymphoma. Lymphocytes are white blood cells that are a very important part of the immune system and helps us fight off infections. B and T cell are the two types of lymphocytes of our immune system. Lymphoma is a type of blood cancer that occurs when these lymphocytes grow in big numbers.
Lymphomas are categorized into Hodgkin lymphomas and Non-Hodgkin lymphomas (NHL).
It is one of the most common types of non-Hodgkin lymphoma. It is known as ‘follicular’ because of development of B cells in the lymph nodes in clumps.
Rituximab has completely changed the outlook of this disease and has significantly improved the overall survival of patients in the last two decades. The average survival is more than 20 years if treatment is taken.
How Common Is Follicular Lymphoma?
Alt tags: Follicular lymphoma
Follicular lymphoma accounts for approximately 30-40% of non-Hodgkin lymphomas. This type of lymphoma is is more commonly seen in older patients over the age of 60. Its occurrence rate in India is at 2.9 per 1 lac for men and 1.5 per lac for women. Studies have shown that its occurrence rates are higher in the Western countries as compared to Asian or African countries.
Follicular lymphoma in children is very rare.
Variants of Follicular Lymphoma
The two types of Follicular Lymphoma are :
1. Duodenal-type follicular lymphoma
This follicular lymphoma type is usually found in the small intestine. It grows slowly and can be diagnosed in its early stages. It responds to treatment very well and doesn’t spread to other parts of the body. Duodenal-type follicular lymphoma is very unlikely to turn into an aggressive form of lymphoma.
2. Pediatric-type follicular lymphoma
As the name depicts, this is a type of lymphoma which affects children and is proven to be very rare in its occurrence. It is usually found in the lymph nodes of head or neck. Similar to duodenal-type follicular lymphoma, this also can be diagnosed in its early stages and responds very well to treatment. Studies have shown very low relapse rates after successful treatment.
What Are The Symptoms Of Follicular Lymphoma?
Its symptoms are not sudden and they present over a period of time. Some people may not develop symptoms at all and its diagnosis may be incidental when tests are performed for some other medical issue.
The most common symptom of follicular lymphoma is swelling of lymph nodes. These enlarged lymph nodes are usually found in the neck area, arm pits and groin area. Over a period of time, the B cell lymphocytes start growing in huge numbers and form a build up and leads to enlargement of lymph nodes. These usually don’t cause any pain to the patient and is one of the first manifestations of the disease.
Below are some more common symptoms associated with follicular lymphoma.
Fatigue (feeling tired all the time)
Weight Loss that cannot be explained and is unintentional
Shortness of breath
Fevers which are sometimes accompanies with night sweats
Pain in the abdomen, accompanied with swelling
Pain in the chest area
Getting sick easily and having a hard time recovering from it
When fever, night sweats and weight loss occurs together, it is known as ‘B Symptoms’.
When follicular lymphoma occurs outside the lymph nodes, it is known as ‘extranodal lymphoma’.
Swollen Lymph Nodes
If the patient is experiencing extreme fatigue and shortness of breath (anemia), it may be attributed to lymphoma cells penetrating the bone marrow. Lymphoma cells in bone marrow may also cause to thrombocytopenia, which is a condition characterized by abnormally low platelet counts. It can also cause neutropenia which is blood condition characterized by abnormally low levels of neutrophils and increases your risk to an array of infections.
Causes of Follicular Lymphoma
The cause of follicular lymphoma largely remains unknown just like other forms of cancer. It is not believed to be genetic and cannot be passed down in families.
Environmental factors may contribute to development and aggravate this form of cancer. It is linked to exposure to deadly substances such as pesticides, dyes, industrial solvents, benzene etc. but the available data is inconsistent and requires further validation. It has also been linked to smoking.
Certain lifestyle factors such as obesity, sedentary lifestyle and unhealthy food are believed to be associated with an increased the risk of follicular lymphoma.
In most of the cases, development of follicular lymphoma cannot be attributed to any cause or risk. At the same time, lots of people with risk factors never go on to develop this condition.
Risk Factors for Follicular Lymphoma
Some factors that are believed to increase the risk of follicular lymphoma are listed below :
Medications that suppress your immune system
Immunosuppressants are prescribed to patients for a variety of diseases, mostly for auto immune conditions and when a patient undergoes transplant. This, in turn compromises the patient’s auto immune system and its ability to fight infections.
Infection with certain viruses and bacteria
Epstein-Barr and HIV are two viruses that are believed to increase the risk of non-Hodgkin’s lymphoma. Influenza vaccination and heart diseases are to also linked with an increased risk but this requires further validation.
Insecticides, pesticides, industrial solvents, benzene etc. may increase your risk of follicular lymphoma.
It is known to occur at all ages, but people over the age of 60 contribute to a significant portion of diagnosed cases. Hence, it is believed that the risk of follicular lymphoma increases with age.
Grading Of Follicular Lymphoma
Follicular Lymphoma is divided into four categories on the basis of number of centroblast (large cells) that are observed. It is common in this type of cancer to get diagnosed at advanced stages when the bone marrow is also infiltered. At the same time, if diagnosed in early stages, it can be cured completely in one third cases with no recurrence.
Stage I: Only one lymph node area or one organ is involved
Stage II – More than two lymph node areas are involved on the same side of diaphragm
Stage III – When cancer is found on both sides of the diaphragm
Stage IV – This stage indicates widespread disease involving multiple regions or organs. When the lymphoma reaches liver or bone marrow, it is always classified as stage IV.
At the time of stage assignment, A or B letter is also included to denote whether the patient has B symptoms (fever, weight loss and night sweats).
Diagnosis and Staging
A physical examination includes feeling lymph nodes in the most common regions where they occur to check if they are enlarged. Neck, armpits and groin regions are usually checked for swollen lymph nodes. The doctor may also check for any signs of swelling in other regions such as throat, spleen and liver, which is usually accompanied by a condition of lymphatic system.
Biopsy is the most important part of diagnosis. A sample is taken from suspected affected lymph node to check for lymphoma cells.
The blood investigations include a complete blood count, kidney, and liver function tests, and signs for any viral infections such as Hepatitis B and C, HIV. Enzyme lactate dehydrogenase (LDH) levels are also checked as part of the investigation since one fourth of the patients with follicular lymphoma have elevated levels of LDH.
A woman undergoing PET scan
CT Scan of neck, chest, abdomen and pelvis area is recommended. PET Scan is useful to identify which areas of the body are affected and stage of the lymphoma.
What Is The Best Treatment of Follicular Lymphoma
The treatment course will be decided by your doctor on the basis of classification of disease stage. Stage I and II are considered as early stages whereas Stage III and IV are considered as advanced stages of lymphoma. The treatment will be influenced by patient’s age, health condition, location of the lymphoma, size of the enlarged nymph nodes and result of investigations.
Treatment of Early-Stage Follicular Lymphoma
Only 1 in every 10 people diagnosed are at an early stage of follicular lymphoma. At this stage, radiotherapy is usually the most preferred treatment. If it is located in only one part of organ of the body, then radiation therapy is considered to be the most suitable and most effective, often curing the disease completely.
A recent study suggested that a combination of radiation therapy and systemic or chemoimmunotherapy is more effective than radiation therapy alone.
In a scenario where radiotherapy is not a suitable treatment choice, observation is considered as a reasonable alternative or monoclonal antibody treatment such as Rituximab is advised.
Treatment of Advanced-Stage Follicular Lymphoma
A man receiving radiation therapy
Most of the patients are at advanced stage at the time of diagnosis. The treatment at this stage has been very successful in recent years. The treatment aims to keep the lymphoma under control with a few side effects. Most people feel better with treatment and sometimes relapse is seen.
If the lymphoma is causing problems, chemotherapy combined with antibody therapy is done.
The most common treatments used are
Bendamustine, also used in the treatment of chronic lymphocytic leukemia. This is used after other treatments have not worked
R-CVP – Rituximab + cyclophosphamide, vincristine, and prednisolone
R-CHOP – Rituximab + cyclophosphamide, doxorubicin, vincristine and prednisolon
Studies have shown that R-CVP and R-CHOP are both very effective in treatment of follicular lymphoma. R-CVP is the preferred choice for younger patients.
The common drugs used in antibody therapy are rituximab and obinutuzumab.
Maintenance treatment of antibody therapy is offered to patients who respond well to chemo-immunotherapy treatment. This is done to slow down the progression of diseases and delay instances of recurrence.
There are several antibody therapies currently being tested for follicular lymphoma.
Stem Cell Transplant
In relapsing forms of follicular lymphoma, a stem cell transplant may be considered. In this procedure, healthy stem cells are transplanted into the bone marrow or blood.
There are two types of stem cell transplants
Autologous transplant : In this procedure, stem cells from your own body are used
Allogeneic transplant : In this procedure, stem cells from a healthy donor are transplanted into your body
Is Follicular Lymphoma Deadly?
Follicular lymphoma patients have a good prognosis and go on to live to for many years without any problems. Most of the treatments available are aimed to keep the disease in control and not cure it.
Survival rates are very difficult to predict as each individual progresses and responds differently to treatment. Even though rare, but sometimes follicular lymphoma transforms into an aggressive form of transformed lymphoma called as diffuse large B-cell lymphoma.
Based on the test results a “prognostic score” is given which helps predict the response to treatment. There are different scoring systems for follicular lymphoma. The score is based on patient’s age, the spread of lymphoma, and the results of blood tests. Some scoring systems take into consideration the genetic changes in the lymphoma cells.
What Is The Survival Rate For Follicular Lymphoma?
The five year survival rates for low risk patients is 91%, medium risk patients 78% and high risk patients 53%.
Lymphoma Support Group India is a group of Lymphoma survivor patients who have come together to offer support to patients who get diagnosed with lymphoma.