About diffuse large B-cell lymphoma (DLBCL)
DLBCL develops when the B-lymphocytes undergo a malignant change. They grow in an uncontrolled way and form collections of tumours in the lymph nodes and other parts of the body. It can affect anywhere that lymph tissue is found. The major sites of lymph tissue are:
Who gets DLBCL?
- DLBCL is the most common subtype of NHL, and NHL is the most common type of lymphoma.
- About 30% of people with NHL will have a subtype of DLBCL
- DLBCL affects up to 2000 Australians every year
Causes of diffuse large B-cell lymphoma (DLBCL)
In most cases, there is no specific cause for DLBCL. There are some risk factors that increase your risk of developing DLBCL. Such as:
- Age – more common in people over 60 years old
- Gender – more common in males
- Ethnicity – More common in people of European origin
- Family history – people who have a parent, sibling or child with NHL
- Past infection with – Epstein-Barr virus, human immunodeficiency virus (HIV) and hepatitis B and C
- A weakened immune system due to inherited conditions – such as primary immunodeficiency or an autoimmune disorder
- Exposure to toxic substances – such as pesticides used in farming
Symptoms of diffuse large B-cell lymphoma (DLBCL)
The most common symptom of DLBCL is painless swelling of the lymph nodes. People usually notice this in their armpits, neck, or groin. The lumps appear, don’t go away, and only seem to get bigger.
One in three people with DLBCL have ‘B symptoms’ at diagnosis. These are a group of symptoms that include:
- Unexplained fever
- Drenching night sweats
- Weightloss without trying
Other symptoms of DLBCL can include:
- Feeling tired for no reason
- Itchy skin
- Pain or full feeling in the abdomen
- Confusion or memory changes
- Chest pain or breathlessness
- Frequent infections
Diagnosis of diffuse large B-cell lymphoma (DLBCL)
DLBCL is diagnosed with a number of tests. You may have some or all these tests before an accurate diagnosis can be made.
Lymph node biopsy
The best test to diagnose DLBCL is a lymph node biopsy. A lymph node biopsy is a procedure that involves taking a sample of tissue from a swollen lymph node and examining it under the microscope. If you have multiple swollen lymph nodes you will only have a biopsy of one of them.
Other tests you will have include:
- Medical history and physical exam
- Blood tests – full blood count (FBC), kidney and liver function, electrolytes
- Genetic tests
These tests help your treatment team work out how far the disease has spread and what other organs it might be affecting. Staging helps determine prognosis and treatment. These tests include:
- Computed tomography (CT) scan
- Positron emission tomography (PET) scan
- Ultrasound
- Bone marrow biopsy
- Lumbar puncture
- Skin biopsy
Staging DLBCL
Staging describes how much the lymphoma has affected your body and how far it has spread. Your DLBCL will be staged as either stage 1, 2, 3 or 4. Your stage of DLBCL will depend on:
- How many areas of the body are affected
- Where the DLBCL is in the body
- Whether the DLBCL has spread to the bone marrow or other parts of the body such as the liver, lungs, skin or bones.
| Stage 1 lymphoma Lymphoma is only in one lymph node area, or one organ such as the thymus, or one area of a single organ outside the lymphatic system. |
| Stage 2 lymphoma Lymphoma affects two or more lymph node areas either above or below the diaphragm* or extends locally from one lymph node area into a nearby organ. |
| Stage 3 – 4 lymphoma Lymphoma is in lymph node areas on both sides of the diaphragm*(stage 3), or the cancer has spread throughout the body beyond the lymph nodes (stage 4). |
*the diaphragm is a large muscle that separates your stomach and chest.
World Health Organization (WHO) classification of DLBCL
The WHO has a system that defines (classifies) the subtypes of DLBCL. The classifications are based on:
- Which cell is originally affected and when it was affected
- What the lymphoma cell looks like under a microscope
- Where the lymphoma cells are found in your body
- Any genetic or chromosome problems (abnormalities)
Here are some more common subtypes of DLBCL
- DLBCL, not otherwise specified (NOS)
- T-cell/histocyte-rich large B-cell lymphoma
- ALK-positive large B-cell lymphoma
- EBV-positive DLBCL
- Primary mediastinal large B-cell lymphoma
- Primary central nervous system lymphoma (PCNSL)
- High grade B-cell lymphoma (previously double hit lymphoma)
Primary central nervous system lymphoma (PCNSL)
- Is a rare and fast-growing type of DLBCL
- It usually affects people between 50 and 60, but it can happen at any age
- It starts in the central nervous system, which includes the brain and spinal cord
- In most cases, it appears in the brain, spinal cord, eye, or the thin layers around the brain and spinal cord (leptomeninges)
- The brain is the most commonly affected area
If the lymphoma starts in another part of the body and spreads to the brain or spinal cord, it’s called secondary CNS lymphoma (SCNSL). PCNSL is treated differently from other types of lymphoma. This is because regular treatments don’t easily reach the brain and spinal cord.
High grade B-cell lymphoma (previously double hit lymphoma)
- Is a rare and aggressive subtype of DLBCL
- There are unusual changes in two genes: MYC and either BCL2 or BCL6, leading to uncontrolled cell growth
- It is difficult to treat because of the genetic mutation
- It has a lower rate of survival compared to other subtypes of DLBCL
- It’s at higher risk of spreading to the CNS
High grade B-cell lymphoma is treated aggressively. You are likely to receive high dose chemotherapy and treatment to prevent the lymphoma reaching the CNS (prophylaxis). Chemotherapy is likely to be given in combination with immunotherapy and sometimes radiotherapy If the lymphoma doesn’t respond to initial treatment, a stem cell transplant may be considered.
Prognosis of diffuse large B-cell lymphoma (DLBCL)
A prognosis is an estimate your haematologist will make of the likely course and outcome of your disease.
Many factors will be considered when discussing your prognosis. Some of these are:
- Your subtype and stage of DLBCL
- Your overall health
- Your age
Your prognosis might change if your DLBCL comes back after treatment or responds poorly to treatment.
Treatment of diffuse large B-cell lymphoma (DLBCL)
The specific treatment you receive for your DLBCL will depend on your subtype. You are likely to receive some chemotherapy in combination with immunotherapy or radiotherapy. You may have some treatment for lymphoma in the CNS, or preventative treatment to stop the lymphoma from spreading to the CNS. Your treatment team will talk to you about stem cell transplant if this is recommended for you.
Some examples of combination treatment are:
- A combination of chemo, immunotherapy and steroids
- Usually given over one day, every 14 or 21 days
- This treatment is given as intravenous infusions and tablets
- You can normally have this treatment in an outpatient clinic
- A combination of chemo, immunotherapy and steroids
- This treatment is given at a different dose depending on your type of DLBCL. The DA stands for ‘dose adjusted’
- It is given as a continuous intravenous infusion over 5 days, every 21 days.
- You usually need to stay in hospital for this treatment
Hyper CVAD
- A combination of high dose chemo and steroids
- Usually given if you have an aggressive high-grade lymphoma
- There are 2 parts to this treatment – Part A and Part B
- It involves complex chemo infusions
- You will be required to stay in hospital for close monitoring
Chemo side effects can vary. Read more about the general side effects here: Chemotherapy – Leukaemia Foundation
Immunotherapy for DLBCL
Immunotherapy is a type of biological therapy. It uses the immune system to recognise cancer cells and destroy them. Immunotherapies for DLBCL can be given intravenously (IV) or subcutaneously (subcut). Immunotherapy works by:
- Stimulating the immune system to find and attack cancer cells
- Removes barriers that are preventing the immune system from killing cancer cells
Monoclonal antibodies are a type of immunotherapy. For people with DLBCL, they are often given in combination with chemotherapy.
Monoclonal anti-CD20 antibody:
Rituximab and Obinutuzumab are examples of monoclonal anti-CD20 antibodies.
- CD20 is a protein found on the surface of B-cells in some subtypes of DLBCL
- This is termed CD20 positive
- The monoclonal anti-CD20 antibody connects to this protein on the blood cancer cell
- It destroys the cell by damaging the antibody, causing the cell to die
Bispecific T-cell Engagers (BiTEs):
Epcoritamab and Glofitamab are examples of a bispecific T-cell engagers used to treat DLBCL
- Combines 2 different monoclonal antibodies
- Attach to 2 different proteins at the same time
- They attach to a protein on cancer cells and to a protein on immune cells
- The immune cells and cancer cells come into contact to help the immune system kill the cancer cells
- These medications are used to treat relapsed or refractory DLBCL
Stem cell transplant
A stem cell transplant is sometimes used to treat people with DLBCL. It is a treatment that restores stem cells after high dose chemotherapy. You may have an autologous stem cell transplant, using your own stem cells. Or, you may have an allogeneic stem cell transplant, using donor stem cells.
Your treatment team will let you know if this is an option for you.
For more information go to: Stem cell transplants – Leukaemia Foundation
Follow-up care
You will need regular checkups with your treatment team once you have finished treatment for your DLBCL. This is to check on any long-lasting side effects from the treatment. You will also need to have tests and scans to make sure the lymphoma hasn’t come back (relapsed/recurred). It is possible you may require more treatment to keep the lymphoma under control if it returns.
Living with DLBCL
How DLBCL affects your everyday life will depend on many factors. It could be that you are returning or managing work, trying to exercise or managing your nutrition. There are some helpful resources and information to guide you – Living well with blood cancer. The Online Blood Cancer Support Service – Leukaemia Foundation has learn modules on long term side effects, transition to the new normal, cancer related fatigue and more.
Caring for someone with diffuse large B-cell lymphoma (DLBCL)
We have a range of information and resources that may help when you are caring for someone with diffuse large B-cell lymphoma (DLBCL).
References
- Other B-cell lymphoma | eviQ
- Diffuse Large B-Cell Lymphoma (DLBCL)
- Large B-Cell Lymphomas in the 5th Edition of the WHO-Classification of Haematolymphoid Neoplasms—Updated Classification and New Concepts – PMC
- Diffuse Large B-cell Lymphoma (DLBCL) – Lymphoma Australia
- High_Grade_B_Cell_Lymphoma_Fact_Sheet_2024.pdf
