Follicular Lymphoma

The Australian Institute of Health and Welfare (AIHW) suggests that about 1500 people will be diagnosed with Follicular Lymphoma in Australia each year. It is the most common subtype of indolent lymphomas.

Follicular Lymphoma (FL)  is a type of blood cancer that changes certain blood cells in your body called B-cell lymphocytes (B-cells). It can affect your lymph nodes (sometimes called glands) and your lymphatic system. These are all part of your body’s immune system which fight infection and disease. Follicular lymphoma is considered an indolent lymphoma, which means it is usually slow growing and often “sleeping”, so many people with FL do not need active treatment during indolent stages of their disease. However, if your FL “wakes up” and starts to grow, you may experience symptoms and need treatment.

There are different subtypes of follicular lymphoma including:

  • Duodenal-type follicular lymphoma (Primary gastrointestinal follicular lymphoma)
  • Paediatric-type follicular lymphoma (childhood)
  • Predominantly diffuse – appearing follicular lymphoma with 1p36 gene deletion.

This webpage will provide you with the information you need if you are getting signs and symptoms, in the process of getting a diagnosis, starting treatment for FL, and common side-effects of treatments for FL.

On this page:

Understanding your B-cell lymphocytes (B-cells)

To understand FL,  you need to know a bit about your B-Cell lymphocytes.

 

B-Cell lymphocytes:

  • Are a type of white blood cell
  • Fight infection and diseases to keep you healthy.
  • Remember infections you had in the past, so if you get the same infection again, your body’s immune system can fight it more effectively and quickly.
  • Are made in your bone marrow (the spongy part in the middle of your bones), but usually live in your spleen and your lymph nodes.  Some live in your thymus and blood too.
  • Can travel through your lymphatic system, to any part of your body to fight infection or disease.
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Follicular lymphoma (FL) develops when your B-cells become cancerous

FL develops when some of your B-cell lymphocytes called follicular centre B-cells become cancerous. When the pathologist looks at your blood or biopsies, under a microscope they will see you have a mixture of centrocyte cells, which are small to medium sized B-cells, and centroblasts which are large B-cells.

Lymphoma occurs when these cells grow uncontrollably, are abnormal, and do not die when they should.

When you have FL the cancerous B-cells:

  • Will not work as effectively to fight infections and disease.
  • Can look different to your healthy B-lymphocyte cells.
  • Can cause lymphoma to develop and grow in any part of your body.

FL is the most common slow growing (indolent) lymphoma and because of the indolent nature of this lymphoma it is routinely found when it is a more advanced stage. Advanced stage FL is not curative, but the goal of treatment is disease control for many years. If your FL is diagnosed in the early stages, you can be cured by certain treatment types.

Very occasionally, Follicular lymphoma (FL) can show a mixture of cells that also include aggressive (fast growing) B-Cell Lymphoma.  This change in behavior can occur over time and is called ‘transformation.  Transformed FL means your cells look and behave more like Diffuse large B cell lymphoma (DLBCL) or rarely, Burkitt’s lymphoma (BL)

Who gets follicular lymphoma (FL)?

FL is the most common subtype of slow growing (indolent) Non- Hodgkin’s Lymphoma (NHL). About 2 out of every 10 people with indolent lymphomas  having a subtype of FL. It is more common in people over 50 years old, and women get it slightly more often than men.

Paediatric follicular lymphoma is rare but can occur in children, teenagers and young adults. It behaves differently to the adult subtype and can often be cured. 

What causes follicular lymphoma?

We don’t know what causes FL, but different risk factors are thought to increase your risk of developing it. Some, risk factors for FL are thought to include: 

  • conditions affecting your immune system such as Celiac disease, Sjogren’s syndrome, lupus, rheumatoid arthritis or human immunodeficiency virus (HIV)
  • prior cancer treatment, with chemotherapy or radiation therapy
  • a family member with lymphoma

*It is important to note, not all people who have these risk factors will develop FL, and some people with none of those risk factors can develop FL.

Patient experience with follicular lymphoma (FL)

Symptoms of follicular lymphoma (FL)

You may not have any symptoms when you are first diagnosed with FL. Many people are only diagnosed when they have a blood test, scans, or a physical examination for something else. This is because of the indolent – slow growing or sleepy nature of FL.

If you do experience symptoms, the first signs and symptoms of FL may be a lump, or several lumps that continue to grow.  You might feel or see them on your neck, armpit, or groin.  These lumps are enlarged lymph nodes (glands), swollen by having too many cancerous B-cells growing in them. They often start in one part of your body, and then spread throughout your lymphatic system.

These lymph nodes can grow very slowly over a long time, which can make it harder to notice if there are any changes. 

Swollen lymph node is often the first symptom of lymphoma

Follicular Lymphoma (FL) can spread to any part of your body

FL can spread to your

  • spleen
  • thymus
  • lungs
  • liver
  • bones
  • bone marrow
  • or other organs.

Your spleen is an organ that filters your blood and keeps it healthy. It is also an organ of your lymphatic system where your B-cells live and make antibodies to fight infection. It is on the left side of your upper abdomen under your lungs and near your stomach (tummy).

When your spleen gets too big, it can put pressure on your stomach and make you feel full, even if you haven’t eaten very much.

Your thymus is also part of your lymphatic system. It is a butterfly shaped organ that sits just behind your breastbone in the front of your chest. Some B-cells also live and pass through your thymus.

General symptoms of lymphoma

Many symptoms of FL can be similar to symptoms found in people any subtype of lymphoma these can include:

  • feeling unusually tired (fatigued)
  • feeling out of breath
  • itchy skin
  • infections that don’t go away or keep coming back
  • changes to your blood tests
    • low red cells and platelets
    • too many lymphocytes and/or lymphocytes that do not work properly
    • lowered white cells (including neutrophils)
    • high lactic acid dehydrogenase (LDH) – a type of protein used to make energy. If your cells are damaged by your lymphoma, LDH can spill out of your cells and into your blood
    • high beta-2 microglobulin – a type of protein made by lymphoma cells. It can be found in your blood, urine or cerebral spinal fluid
  • B-symptoms
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B-symptoms are a group of symptoms that occur together, including night sweats, fever and weight loss. Contact your doctor immediately if you are getting these symptoms.

Other symptoms of follicular lymphoma may depend on where your disease is located in your body

Area affected

Symptoms

Gut – including your stomach and bowel

Nausea with or without vomiting (feeling sick in your tummy or throwing up)

Diarrhoea or constipation (watery or hard poo)

Blood when you go to the toilet

Feeling full even if you haven’t eaten much

Central nervous system (CNS) – including your brain and spinal cord

Confusion or memory changes

Personality changes

Seizures

Weakness, numbness, burning or pins and needles in your arms and legs

Chest

Shortness of breath

Chest pain

A dry cough

Bone marrow

Low blood counts including red cells, white cells and platelets resulting in:

o    Shortness of breath

o    Infections that deep coming back or are difficult to get rid of

o    Unusual bleeding or bruising

 

Skin

Red or purple looking rash

Lumps and Bumps on your skin which may be skin coloured or red or purple

Itching

When to contact your doctor

There are some symptoms that may suggest your FL is starting to grow or become more aggressive. If you experience any of the below symptoms, contact your doctor. Do not wait for next appointments. It’s important to let them as soon as possible so they can make plans for treatment if you may end up needing it.

Contact your if you:

  • have swollen lymph nodes that do not go away, or if they are larger than you would expect for an infection
  • are often short of breath without reason
  • feel more tired than usual and it does not get better with rest or sleep
  • notice unusual bleeding or bruising (including in our poo, from your nose or gums)
  • develop an unusual rash (a purple of red spotty rash can mean you have some bleeding under your skin)
  • get more itchy than usual
  • develop a new dry cough
  • experience B symptoms.

It’s important to note that many of the signs and symptoms of FL can be related to causes other than cancer. For example, swollen lymph nodes can also happen if you have an infection. Usually though, if you have an infection, the symptoms will improve, and the lymph nodes will return to normal size in a few weeks. With lymphoma, these symptoms will not go away. They may even get worse.

How is follicular lymphoma (FL) diagnosed?

Diagnosing FL can sometimes be difficult and can take several weeks.

If your doctor thinks that you may have lymphoma, they will need to organise several important tests. These tests are needed to either confirm or rule out lymphoma as the cause for your symptoms. Confirming the type of Non- Hodgkin’s Lymphoma (NHL) is very important as the management and treatment of your subtype may be different to other subtypes of NHL.

To diagnose FL you will need a biopsy.  A biopsy is a procedure to remove part, or all of an affected lymph node and/ or a bone marrow. The biopsy is then checked by scientists in a laboratory to see if there are changes that help the doctor diagnose FL.

When you have a biopsy, you may have a local or general anaesthetic. This will depend on the type of biopsy and  what part of your body it is taken from. There are different types of biopsies, and you may need more than one to get the best sample.

Blood tests

You will have many blood tests over time. You will start with blood tests even before you get a diagnosed with FL. You will also have them before and during treatment if you need treatment. They give your doctor a picture of your general health, so they can make the best decisions with you about your health care needs and treatment.

Fine needle or core biopsy

A core biopsy involves the doctor using a needle and inserting it into your swollen lymph node or lump so they can remove a sample of tissue to test for lymphoma. This is usually done under a local anaesthetic, while you are awake.   

If the affected lymph node is deep inside you body, the biopsy may be done with the help of ultrasound or specialised x-ray (imaging) guidance. 

Some biopsies may be done with the help of ultrasound guidance
Excisional node biopsy 

An excisional biopsy is done if your swollen lymph nodes are too deep to be reached with a needle, or if your doctor wants to remove and check the whole lymph node.

It will usually be done as day procedure in an operating theatre and you will have a general anaesthetic to put you to sleep for a little while while the procedure is done. When you wake up you will have a small wound and stitches. Your doctor or nurse will be able to let you know how to care for the wound, and when to get your stitches out. 

The doctor will choose the best biopsy for you.

Results

Once your doctor gets the results from your blood tests and biopsies they will be able to tell you if you have FL and may also be able to tell you what subtype of FL you have. They will then want to do more tests to stage and grade your FL.

Staging and grading follicular lymphoma

Once you have been diagnosed with FL, your doctor will have more questions about your lymphoma. These will include:

  • What stage is your lymphoma?
  • What grade is your lymphoma?
  • What subtype of FL do you have?

Click on the headings below to learn more about staging and grading.

Staging refers to how much of your body is affected by your lymphoma – or, how far it has spread from where it first started.

B-cells can travel to any part of your body. This means that lymphoma cells (the cancerous B-cells), can also travel to any part of your body. You will need to have more tests done to find this information. These tests are called staging tests and when you get results, you will find out if you have stage one (I), stage two (II), stage three (III) or stage four (IV) FL.

Your stage of FL will depend on:

  • How many areas of your body have lymphoma
  • Where the lymphoma is including if it is above, below or on both sides of your diaphragm (a large, dome-shaped muscle under the rib cage that separates the chest from your abdomen)
  • Whether the lymphoma has spread to your bone marrow or other organs such as the liver, lungs, skin or bone.

Stages I and II are called ‘early or limited stage’ (involving a limited area of your body).

Stages III and IV are called ‘advanced stage’ (more widespread).

Staging of lymphoma
Stage 1 and 2 lymphoma are considered early stage, and stage 3 and 4 are considered advanced stage lymphoma.
Stage 1

One lymph node area is affected, either above or below the diaphragm*.

Stage 2

Two or more lymph node areas are affected on the same side of the diaphragm*.

Stage 3

At least one lymph node area above and at least one lymph node area below the diaphragm* are affected.

Stage 4

Lymphoma is in multiple lymph nodes and has spread to other parts of the body (e.g. bones, lungs, liver).

Diaphragm
Your diaphragm is a dome shaped muscle that separates your chest and your abdomen.

Extra staging information

Your doctor may also talk about your stage using a letter, such as A,B, E, X or S. These letters give more information about the symptoms you have or how your body is being affected by the lymphoma. All this information helps your doctor find the best treatment plan for you. 

Letter
Meaning
Importance

A or B

  • A = you have no B-symptoms
  • B  = you have B-symptoms
  • If you have B symptoms when you are diagnosed, you may have a more advanced-stage disease.
  • You may still be cured or go into remission, but you will need more intensive treatment

E & X

  • E = you have early stage (I or II) lymphoma with an organ outside of the lymph system – This might include your liver, lungs, skin, bladder or any other organ 
  • X = you have a large tumour that is larger than 10cm in size. This is also called “bulky disease”
  • If you have been diagnosed with limited stage lymphoma, but it is in one of your organs or is considered bulky, your doctor may change your stage to an advanced stage.
  • You may still be cured or go into remission, but you will need more intensive treatment

S

  • S = you have lymphoma in your spleen
  • You may need to have an operation to remove your spleen

(Your spleen is an organ in your lymphatic system that filters and cleans your blood, and is a place your B-cells rest and make antibodies)

Tests for staging

To find out what stage you have, you may be asked to have some of the following staging tests:

Computed tomography (CT) scan

These scans takes pictures of the inside of your chest, abdomen or pelvis. They provide detailed pictures that provide more information than a standard X-ray.

Positron emission tomography (PET) scan 

This is a scan that takes pictures of the inside of your whole body. You will be given and needle with some medicine that cancerous cells – such as lymphoma cells absorb. The medicine that helps the PET scan identify where the lymphoma is and the size and shape by highlighting areas with lymphoma cells. These areas are sometimes called “hot”.

Lumbar puncture

A lumbar puncture is a procedure done to check if you have any lymphoma in your central nervous system (CNS), which includes your brain, spinal cord and an area around your eyes. You will need to say very still during the procedure, so babies and children may have a general anaesthetic to put them to sleep for a little while the procedure is done. Most adults will only need a local anaesthetic for the procedure to numb the area.

Your doctor will put a needle into your back, and take out a little bit of fluid called “cerebral spinal fluid” (CSF) from around your spinal cord. CSF is a fluid that acts a bit like a shock absorber to your CNS. It also carries different proteins and infection fighting immune cells such as lymphocytes to protect your brain and spinal cord. CSF can also help drain any extra fluid you may have in your brain or around your spinal cord to prevent swelling in those areas.

The CSF sample will then be sent to pathology and checked for any signs of lymphoma.

Bone marrow biopsy
A bone marrow biopsy is done to check if there is any lymphoma in your blood or bone marrow. Your bone marrow is the spongey, middle part of your bones where your blood cells are made. There are two samples the doctor will take from this space including:
 
  • Bone marrow aspirate (BMA): this test takes a small amount of the liquid found in the bone marrow space.
  • Bone marrow aspirate trephine (BMAT): this test takes a small sample of the bone marrow tissue.
bone marrow biopsy to diagnose or stage lymphoma
A bone marrow biopsy can be done to help diagnose or stage lymphoma

The samples are then sent to pathology where they are checked for signs of lymphoma.

The process for bone marrow biopsies can differ depending on where you are having your treatment, but will usually include a local anaesthetic to numb the area.

In some hospitals, you may be given light sedation which helps you to relax and can stop you from remembering the procedure. However many people do not need this and may instead have a “green whistle” to suck on. This green whistle has a pain killing medication in it (called Penthrox or methoxyflurane), that you use as needed throughout the procedure.

Make sure you ask your doctor what is available to make you more comfortable during the procedure, and talk to them about what you think will be the best option for you.

More information on bone marrow biopsies can be found at our webpage here.

Your lymphoma cells have a different growth pattern and look different to normal cells.  The grade of your Follicular lymphoma is how your lymphoma cells look under a microscope. Grades 1-2 (low grade) have a small number of centroblasts (large B-cells).  Grades 3a and 3b (high grade) have a larger number of centroblasts (large B-cells), and often centrocytes (small to medium B cells) are seen as well.  Your cells will look different to normal cells and grow differently. The more centroblast cells present the more aggressive (fast growing) your tumour will be. An overview of the grades is below.

World Health Organisation (WHO) grading of follicular lymphoma (FL)

Grade

Definition

1

Low grade: 0-5 centroblasts seen in the lymphoma cells. 3 out of 4 cells are indolent (slow growing) follicular B-cells

2

Low grade: 6-15 centroblasts seen in the lymphoma cells.  3 out of 4 cells are indolent (slow growing) follicular B-cells

3A

High grade: More than 15 centroblasts and also centrocytes present in the lymphoma cells. There is a mix of indolent (slow growing) follicular lymphoma cells and aggressive (fast growing) lymphoma cells called diffuse large B cells.

3B

High grade: More than 15 centroblasts with NO centrocytes seen in the lymphoma cells. There is a mix of indolent (slow growing) follicular lymphoma cells and aggressive (fast growing)  lymphoma cells called diffuse large B cells. because of this Grade 3b is treated as Diffuse Large B Cell Lymphoma subtype (DLBCL) ADD: Link to DLBCL

The grading and staging of your FL are very important as it indicates whether you need treatment and what type of treatment.

  • Stage IV FL may not require treatment immediately and you may be put on to active monitoring (watch and wait) as you have low grade (slower growing) FL.
  • Grade FL- 3A and 3B it is routinely treated similarly to DLBCL which is a more aggressive subtype of NHL.

It is important that you talk to your doctor about your own risk factors so you can have clear idea of what to expect from your treatments.

Subtypes of Follicular lymphoma (FL)

Once your doctor gets all your results back, they will be able to let you know what stage and grade of FL you have. You may also be told you have a specific subtype of FL, but this is not the case for everyone.

If you are told you have a specific subtype, click on the links below to learn more about that subtype. 

Duodenal-type follicular lymphoma is also called primary gastrointestinal follicular lymphoma (PGFL). It is a very slow-growing FL and is often diagnosed in its early stages. 

It grows in the first portion of your small intestine (duodenum), just past your stomach. PGFL is mostly localised, which means it is only found in one place, and usually does not spread to other parts of your body.

Symptoms

Some symtoms you may have with PGFL include  abdominal pain and heartburn, or you may experience no symptoms at all.  Treatment may be surgery or watch and wait (active monitoring). depending on your symptoms.

Even when surgery is needed, the outcome for people with Duodenal-type FL is very good.

Predominantly diffuse appearing FL is a group of scattered (diffuse) lymphoma cells that are mainly found in one part of your body. The main symptoms is a large mass (tumour) that appears as a lump in your groin (inguinal) area. 

Paediatric-type follicular lymphoma is a very rare form of follicular lymphoma. It mostly affects children but can also affect adults up to about 40 years of age. 

Research shows that P-TFL is unique and different to standard follicular lymphoma. It behaves more like a benign (non cancerous) tumour and is usually found in only in one part of your body. It does not usually spread away from the area it first grows.

PTFL is most common in the lymph nodes near your head and neck.

Treatment for paediatric-type follicular lymphoma may involve surgery to remove the affected lymph nodes, or watch & wait (active monitoring). After successful treatment, this subtype rarely comes back.

Understanding your lymphoma cytogenetics

As well as all the above tests, you may also have cytogenetic tests. This is where your blood and tumour sample are checked for genetic variances that may be involved in your disease. For more information on these please see our section on understanding your lymphoma genetics further down on this page. The tests used to check for any genetic mutations are called cytogenetic tests. These tests look to see if you have any changed in chromosomes and genes.

We usually have 23 pairs of chromosomes, and they are numbered according to their size. If you have FL, your chromosomes may look a little different.  

 

Changes in your genes and chromosomes can help work out your diagnosis, and can affect your treatment options
What are genes and chromosomes?

Each cell that makes up our body has a nucleus, and inside the nucleus are the 23 pairs of chromosomes. Each chromosome is made from long strands of DNA (deoxyribonucleic acid) that contain our genes. 

Our genes provide the code needed to make all the cells and proteins in our body and tells them how to look or act. 

If there is a change (variation) in these chromosomes or genes, your proteins and cells will not work properly. 

Lymphocytes can become lymphoma cells due to genetic changes (called mutations or variations) within the cells. Your lymphoma biopsy may be looked at by a specialist pathologist to see if you have any gene mutations.

What do FL mutations look like?

Overexpression

Research has found different genetic changes (mutations) can cause an overexpression (too much) of certain proteins on the surface of FL cells. When these proteins are overexpressed, they help your cancer grow.

The different proteins are part of a group that normally tell cells to grow, or die and keep a healthy balance. They also usually recognise if a cell becomes damaged, or starts to become cancerous, and tell these cells to either fix themselves, or die. But the overexpression of some of the proteins telling the lymphoma cells to grow, causes this process to become unbalanced, and allow the cancerous cells to keep growing and multiplying. 

Some proteins that may be overexpressed on your FL cells include:

  • CD5
  • CD10
  • CD20
  • CD23
  • CD43
  • BCL6
  • IRF4
  • MUM1

Translocation

Genes can also cause activity and growth changes because of a translocation. A translocation occurs when genes on two different chromosomes swap places. Translocations are quite common in people with FL. If you have a translocation in your FL cells, it is likely to be between your 14th and 18th chromosome.  When you have a translocation of genes on the 14th and 18th chromosomes it is written as t(14:18)

Why is it important to know about what genetic changes I have?

Genetic changes are important because they can help your doctor predict how your FL will act and grow. It also helps them plan what tratment might work best for you.

Remembering the name of your genetic changes isn’t so important. But, knowing you have some of these gene mutations explains why you may need different treatment or medication to others with FL. 

The discovery of genetic changes in lymphoma has led to research and development of new treatments that target the proteins or genes involved. This research is ongoing as more changes are found.

Some ways your genetic change can impact your treatment include:

  • If CD20 is overexpressed on your FL cells and you need treatment, you may have a medication called rituximab (also called Mabthera or Rituxan). CD20 overexpression is very common in people with follicular lymphoma.
  • If you have an overexpression of IRF4 or MUM1, it may indicate that your FL is more aggressive than indolent, and may require treatment.
  • Some genetic changes may mean that targeted therapy will be more effective to treat your FL.

Questions to ask your doctor

It can be difficult to know what questions to ask when you are starting treatment. If you don’t know, what you don’t, how can you know what to ask?

Having the right information can help you feel more confident and know what to expect. It can also help you plan ahead for what you may need.

We put together a list of questions you may find helpful. Of course, everyone’s situation is unique, so these questions do not cover everything, but they do give a good start. 

Click on the link below to download a printable PDF of questions for your doctor

Treatment for Follicular Lymphoma (FL)

Once all the results from your biopsies, cytogenetic testing and staging scans have come in, your doctor will be able to make plans about how to manage your FL. In many cases this may mean taking “Watch and Wait” approach. This means that your lymphoma does not need any treatment, but they will want to keep a close watch to see if the lymphoma begins to grow more, or cause you to have symptoms or become unwell. You can download our factsheet on Watch and Wait by clicking the link below.

When to start treatment

Your doctor will review these to decide the best possible treatment for you.   At some cancer centres, the doctor will also meet with a team of specialists to discuss the best treatment option. This is called a multidisciplinary team (MDT) meeting.  

Your doctor will consider many factors about your FL. Decisions on when or if you need to start and what treatment is best are based on:

  • Your individual stage of lymphoma, genetic changes, and symptoms 
  • Your age, past medical history, and general health
  • Your current physical and mental wellbeing and patient preferences. 

More tests may be ordered before you start treatment to make sure your heart, lungs and kidneys are able to cope with the treatment. These may include an ECG (electrocardiogram), lung function test or 24-hour urine collection. 

Your doctor or cancer nurse can explain your treatment plan and the possible side-effects to you and are there to answer any question you might have. It is important you ask your doctor and/or cancer nurse questions about anything you don’t understand.

The aim of treating FL is to:
  • Prolong remission
  • Provide disease control
  • Improve quality of life
  • Reduce symptoms or side effects with supportive or palliative care

You can also phone or email the Lymphoma Australia Nurse Helpline with your  questions and we can help you to get the right information. 

Lymphoma care nurse hotline:

Phone: 1800 953 081

Email: nurse@lymphoma.org.au

When is treatment needed for Follicular Lymphoma (FL)?

As mentioned above, not everyone with FL will need to start treatment right away. To help your doctors determine when it’s time to start treatment a criteria called the ‘GELF criteria’ was established. If you have one or more of these symptoms, it is likely you may need treatment:

  • A tumour mass larger than 7cm in size.
  • 3 swollen lymph nodes in 3 distinctly different areas, all bigger than 3cms in size.
  • Persistent B symptoms.
  • Enlarged spleen (splenomegaly)
  • Pressure on any of your internal organs as a result of swollen lymph nodes. 
  • Fluid with lymphoma cells in either your lungs or abdomen (Pleural effusions or ascites).
  • FL cells found in your blood or bone marrow (leukemic changes) or a decrease in your other health blood cells (cytopenias). This means your FL is stopping your bone marrow from being able to produce enough health blood cells.
  • Elevated LDH or Beta2- microglobulin (these are blood tests).

Click on the below headings to see the different types of treatment that may be used to manage your FL.

Supportive care is given to patients and families facing serious illness. Supportive care can help patients have fewer symptoms, and actually get better faster by paying attention to those aspects of their care.

For some of you with FL, your leukemic cells may grow uncontrollably and crowd your bone marrow, bloodstream, lymph nodes, liver or spleen. Because the bone marrow is crowded with FL cells too young to work properly, your normal blood cells will be affected. Supportive treatment may include things like you having blood or platelet transfusions on a ward or in an intravenous infusion suite in the hospital. You may have antibiotics to prevent or treat infections.

It may involve a consultation with a specialised care team or even palliative care. It can also be having conversations about future care, which is called Advanced Care Planning. These things are part of multidisciplinary management of lymphoma.

Supportive care can include palliative care which helps to improve your symptoms and side-effects, as well as end of life care if needed

It is important to know that the Palliative Care team can be called on at any time during your treatment pathway not just at the end of life. They can help control and manage symptoms (like hard to control pain and nausea) you might be experiencing as a result of your disease or its treatment. 

If you and your doctor decide to use supportive care or stop curative treatment for your lymphoma, many things can be done to help you to stay as healthy and comfortable as possible for some time. 

Radiation therapy is a cancer treatment that uses high doses of radiation to kill lymphoma cells and shrink tumours. Before having radiation, you will have a planning session. This session is important for the radiation therapists to plan how to target the radiation to the lymphoma, and avoid damaging healthy cells. Radiation therapy usually lasts between 2-4 weeks. During this time, you will need to go to the radiation centre everyday (Monday-Friday) for treatment. 

*If you live a long way from the radiation centre and need help with a place to stay during treatment, please talk to your doctor or nurse about what help is available to you. You can also contact the Cancer Council or Leukemia Foundation in your state and see if they can help with somewhere to stay.

Radiation treatment for lymphoma
Radiation can be used to treat early stage lymphoma, or to improve symptoms by shrinking your tumour

 

You might have these medications as a tablet and/ or be given as a drip (infusion) into your vein (into your bloodstream) at a cancer clinic or hospital. Several different chemo medications may be combined with an immunotherapy medicine. Chemo kills fast growing cells so can also affect some of your good cells that grow fast causing side effects.

You may have a MAB infusion at a cancer clinic or hospital. MABs attach to the lymphoma cell and attract other diseases fighting white blood cells and proteins to the cancer so your own immune system can fight the FL.

MABS will only work if you have specific proteins or markers on your lymphoma cells. A common marker in FL is CD20. If you have this marker you may benefit from treatment with a MAB.

Chemotherapy combined with a MAB (for example, rituximab).

You may take these as a tablet or infusion into your vein. Oral therapies may be taken at home, though some will require a short hospital stay. If you have an infusion, you may have it at a day clinic or in a hospital. Targeted therapies attach to the lymphoma cell and block signals it needs to grow and produce more cells. This stops the cancer from growing and causes the lymphoma cells to die off. 

A stem cell or bone marrow transplant is done to replace your diseased bone marrow with new stem cells that can grow into new healthy blood cells. Bone marrow transplants are usually only done for children with FL, while stem cell transplants are done for both children adults.

In a bone marrow transplant, stem cells are removed straight from the bone marrow, where as with a stem cell transplant, the stem cells are removed from the blood.

The stem cells may be removed from a donor or collected from you after you’ve had chemotherapy.

If you the stem cells come from a donor, it is called an allogeneic stem cell transplant.

If your own stem cells are collected, it is called an autologous stem cell transplant.

Stem cells are collected through a procedure called apheresis. You (or your donor) will be connected up to an apheresis machine and your blood will be removed, the stem cells separated and collected into a bag, and then the rest of your blood is returned to you.

Before the procedure, you will get high-dose chemotherapy  or  full-body radiotherapy to kill off all your lymphoma cells. However this high dose treatment will also kill off all the cells in your bone marrow. So the collected stem cells will then be returned to you (transplanted). This happens in much the same was as blood transfusion is given, through a drip into your vein.

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CAR T-cell therapy is a newer treatment that will only be offered if you have already had at least two other treatments for your FL.

In some cases, you may be able to access CAR T-cell therapy by joining a clinical trial. 

CAR T-cell therapy involves an initial procedure similar to a stem cell transplant, where your T-cell lymphocytes are removed from you blood during an apheresis procedure. Like you B-cell lymphocytes, T-cells are part of your immune system and work with your B-cells to protect you from disease and illness.

When the T-cells are removed, they are sent to a laboratory where they are re-engineered. This happens by joining the T-cell to an antigen that helps it recognise the lymphoma more clearly and fight it more effectively.

Chimeric means having parts with different origins so the joining of an antigen to the T-cell makes it chimeric.

Once the T-cells have been re-engineered they will be returned to you to start fighting the lymphoma.

First-line treatment - Starting treatment

Starting Therapy

The first time you start treatment, it’s called first-line treatment. Once you finish your first-line treatment, you may not need treatment again for many years. Some people need more treatment straight away, and others may go months or years, before needing more treatment.

When you do start treatment, you may have more than one medicine. This may include chemotherapy, a monoclonal antibody or targeted therapy. In some cases, you may have radiation treatment or surgery as well, or instead of medications.

Treatment cycles

When you have these treatments, you will have them in cycles. That means you will have the treatment, then a break, then another round (cycle) of treatment. For most people with FL, chemoimmunotherapy is effective to achieve a remission (no signs of cancer).  

When your whole treatment plan is put together, it is called your treatment protocol. Some places may call it a treatment regime. 

Starting treatment for lymphoma or CLL can make you worried

Your doctor will choose the best treatment protocol for you based on the below factors

  • The stage and grade of your FL.
  • Any genetic changes you have.
  • Your age and overall health.
  • Other illnesses or medications you may be taking.
  • Your preferences after discussing your options with your doctor.

Examples of chemoimmunotherapy protocols you may get to treat FL

  • BR a combination of Bendamustine and Rituximab (a MAB).
  • BO or GB- a combination of Bendamustine and Obinutuzumab (a MAB).
  • RCHOP a combination of rituximab (a MAB) with chemotherapy medicines cyclophosphamide, doxorubicin, vincristine and prednisolone. This protocol is only used to treat FL when it is higher grade, commonly grade 3a and above.
  • O- CHOP a combination of Obinutuzumab, cyclophosphamide, vincristine, doxorubicin and prednisolone. This protocol is only used to treat FL when it is higher grade, commonly grade 3a and above.

Clinical trials

There are many clinical trials across Australia and around the world, looking for ways to improve the treatment for people with lymphoma. If you are interested in learning more about clinical trials, click the button below. You can also talk to your specialist doctor – your haematologist or oncologist about what clinical trials you may be eligible for.

Maintenance therapy

Maintenance therapy is given with the purpose of keeping you in remission for a longer time, after your first-line treatment.

Complete remission

Many people have a very good response to first-line treatment and achieve complete remission. This means that when you finish your treatment, there is no detectable FL left in your body. This can be confirmed after a PET scan. However it’s important to understand that a complete remission is not the same as a cure. With a cure, the lymphoma is gone and is not likely to come back.

But we know with indolent lymphomas such as FL, they often come after some time. This may be months or years after your treatment, but it is still likely to come back. This is called a relapse. When it happens, you may need more treatment, or you may go onto “watch and wait” if it remains indolent with no symptoms.

Partial remission

For some people, first-line treatment does not result in a complete remission, but rather a partial remission. This means most of the disease has gone, but there are still some signs of it left in your body. It is still a good response, because remember FL is an indolent lymphoma that cannot be cured. But if you have a partial response, it may go back to sleep, and you may not need anymore active treatment but go on to watch and wait.

Whether you have complete or partial remission can be seen on your follow up PET scan. 

To try to keep you in remission for as long as possible, your doctor may suggest you go onto maintenance therapy for two years after your first-line treatment.

What does maintenance therapy involve?

Maintenance therapy is usually given once every 2-3 months and is a monoclonal antibody. The monoclonal antibodies used for maintenance are rituximab or obinutuzumab. Both of these medications are effective when you have the protein CD20 on your lymphoma cells, which is common with FL.

Second-line Treatment

If your FL relapses or is refractory to first-line treatment, you may need a second-line treatment. Refractory FL is when you do not have a complete or partial remission from your first-line treatment. 

If you under 70 years of age, you may be offered different combinations of medications, followed by a stem-cell transplant. However, stem cell transplants are not suitable for everyone. Your doctor will be able to talk to you more about your individual suitability for this treatment type. 

If you do not have a stem-cell transplant, there are other treatment protocols you may be offered. 

These treatments are used to get you back into a remission and control your lymphoma longer term. 

Treatment protocols if you having a stem-cell transplant

 RICE

RICE is an intense chemo of fractionated (broken up) or infusional (through a drip) doses of  ifosfamide, carboplatin and etoposide. You may have this if you have relapsed or prior to an autologous stem cell transplant. You will need to have this treatment in hospital

 R-GDP 

R-GDP is a combination of gemcitabine, dexamethasone and cisplatin. You may have this if you have relapsed or prior to an autologous stem cell transplant.

Treatment protocols if you are not having a stem-cell transplant

R-CHOP/ O-CHOP

R-CHOP or O-CHOP is a combination of rituximab or obinutuzumab (a MAB)  with chemo medicines cyclophosphamide, doxorubicin, vincristine and prednisolone link to eviQ.

R-CVP

R-CVP is a combination of rituximab, cyclophosphamide, vincristine and prednisolone. You may have this if you are older with other health issues.

O-CVP

O-CVP is a combination of obinutuzimab, cyclophosphamide, vincristine and prednisolone. You may have this if you are older with other health issues.

Radiation

Radiation therapy can be used when your FL relapses. It is usually done if it relapses in one local area and helps to control your FL and minimise some symptoms you may be getting.  

Third-line treatment

In some cases, you may need more treatment after a second or even third relapse. Third-line treatment will often be similar to the treatments above.

In some cases, if your FL is “transforming” and starting to behave more like an aggressive subtype of lymphoma called Diffuse large B-cell lymphoma, you may be eligible for CAR T-cell therapy as a third or fourth-line treatment. Your doctor will let you know if your FL begins to transform.

Transformed Lymphoma

A transformed lymphoma is a lymphoma that was initially diagnosed as indolent (slow growing) but has become (transformed into) an aggressive (fast growing) lymphoma.

Transformation of your FL may happen if you have more genetic changes to your lymphoma cells over time, causing further damage. This can happen naturally, or as a result of some treatments. This extra damage to the genes causes the cells to grow faster. 

The risk of a transformation is low. Research shows that over the 10 to 15 years after diagnosis, about 2-3 people out of 100 with FL each year, may have a transformation to a more aggressive subtype.

The average time from a diagnosis to transformation is 3-6 years.

If you have a transformation from FL, it is likely that it will transform into a subtype of lymphoma called Diffuse large B-cell lymphoma (DLBCL) or, more rarely Burkitt lymphoma. You will need chemoimmunotherapy treatment straight away.

Due to advances in treatments, the outcome for transformed follicular lymphoma has greatly improved over recent years.  

Common side-effects of treatment

There are many different side effects you can get from your treatment for FL. Before you start your treatment, your doctor or nurse should explain all the expected side-effects you MAY experience. You may not get all of them, but it is important to know what to look out for and when to contact your doctor. Make sure you have the contact details of who you should contact if you become well in the middle of the the night or on the weekend when your doctor may not be available. 

One of the most common side-effects of treatment is changes to your blood counts. Below is a table that describes which blood cells may be affected and how that may affect you.

Blood cells affected by FL treatment

 

White blood cells

Red blood cells

Platelets (also blood cells)

Medical Name

Neutrophils & Lymphocytes

Erythrocytes

Platelets

What do they do?

Fight Infection

Carry oxygen

Stop bleeding

What is a shortage called?

Neutropenia & lymphopenia

Anaemia

Thrombocytopenia

How will this affect my body?

You will get more infections and may have difficulty getting rid of them even with taking antibiotics

You may have pale skin, feel tired, breathless, cold and dizzy

You may bruise easily, or have bleeding that doesn’t stop quickly when you have a cut

What will my treating team do to fix this?

●        Delay your lymphoma treatment

●        Give you oral or intravenous antibiotics if you have an infection

●          Delay your lymphoma treatment

●          Give you a red cell blood transfusion if your cell count is too low

●        Delay your lymphoma treatment

●        Give you a platelet transfusion if your cell count is too low

When all of these blood cells are low, it is called pancytopenia. If you are pancytopenic, your doctor may want to admit you to the hospital for treatment until your counts are at a safer level. 

Other common side-effects of treatment for FL

Below is a list of some other common side-effects of FL treatments. It is important to note that now all treatments will cause these symptoms, and you should talk to your doctor or nurse about which side-effects may be caused your individual treatment.

  • Feeling sick in the stomach (nausea) and vomiting.
  • Sore mouth (mucositis) and change to the taste of things.
  • Bowel problems such as constipation or diarrhoea (hard or watery poo).
  • Tiredness, or a lack of energy that doesn’t get better after a rest or sleep (fatigue).
  • Muscle (myalgia) and joint (arthralgia) aches and pains.
  • Hair loss and thinning (alopecia) – only with some treatments.
  • Mind fogginess and difficulty in remembering things (chemo brain).
  • Changed sensation in your hands and feet such as tingling, pins and needles or pain (neuropathy).
  • Reduced fertility or early menopause (change of life).

Follow up care - What happens when treatment ends?

When you finish treatment, you may want to throw on your dancing shoes, put your arms in the air and party like this guy (if you have the energy), or you may be filled with worry and stress about what comes next.

Both feelings are common and normal. It’s also normal to feel one way, one moment, and the other way the next moment.

You are not alone when treatment ends. You will still have regular contact with your specialist team, and be able to call them if you have any concerns. 

You will still be monitored with blood test and scans to check for any signs or relapse or long lasting side-effects from your treatment.

Prognosis

Prognosis is the term used to describe the likely path of your disease, how it will respond to treatment and how you will do during and after treatment. 

There are many factors that contribute to your prognosis and it is not possible to give an overall statement about prognosis. However, FL often responds very well to treatment and many patients with this cancer can be cured – meaning after treatment, there is no sign of FL in your body.

Factors that can impact prognosis

Some factors that may impact your prognosis include:

  • You age and overall health at time of diagnosis.
  • How you respond to treatment.
  • What if any genetic mutations you have.
  • The subtype of FL you have.

If you would like to know more about your own prognosis, please talk with your specialist haematologist or oncologist. They will be able to explain your risk factors and prognosis to you.

Survivorship - living with follicular lymphoma

A healthy lifestyle, or some positive lifestyle changes after treatment can be a great help to your recovery.  There are many things you can do to help you live well with DLBCL. 

Many people find that after a cancer diagnosis, or treatment, that their goals and priorities in life change. Getting to know what your ‘new normal’ is can take time and be frustrating. Expectations of your family and friends may be different to yours. You may feel isolated, fatigued or any number of different emotions that can change each day.

The main goals after treatment for your DLBCL is to get back to life and:            

  • Be as active as possible in your work, family, and other life roles.
  • Lessen the side effects and symptoms of the cancer and its treatment.  
  • Identify and manage any late side effects.      
  • Help keep you as independent as possible.
  • Improve your quality of life and maintain good mental health.

Different types of cancer rehabilitation may be recommended to you. This could mean any of a wide range of services such as:     

  • Physical therapy, pain management.      
  • Nutritional and exercise planning.      
  • Emotional, career and financial counselling. 

Extra resources for you

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