Non-Hodgkin Lymphoma

The lymphatic system is part of the immune system and includes your lymph glands and organs such as the spleen. Its functions include:¹

• Maintaining fluid balance and removal of cellular waste
• Absorbing and transportation of nutrients
• Production of immune cells to help fight infections

The lymphatic system contains white blood cells called lymphocytes. Non-Hodgkin lymphoma develops in these cells, causing them to grow abnormally. These abnormal lymphocytes most commonly accumulate in the lymph nodes.¹ 

Although lymph nodes are the most frequently affected sites, non-Hodgkin lymphoma can also involve the spleen and other extranodal sites, including the central nervous system, bone and stomach.¹ 

Non-Hodgkin lymphoma can cause different symptoms depending on where it develops in the body. Symptoms may include:¹

• Persistent, unexplained fevers
• Night sweats that soak clothing or bedding
• Rapid, unintentional weight loss

'B symptoms'

Some symptoms are known as B symptoms. These can help doctors assess how advanced the lymphoma may be. They include: (1)

• Painless swelling of the lymph nodes, most commonly in the neck, armpit or groin
• Fever
• Excessive night sweats
• Unexplained weight loss
• Itchy skin or skin rashes
• Abdominal swelling or back pain
• Headaches or double vision
• Cough, difficulty swallowing, or shortness of breath

Many other conditions can cause these symptoms.¹ If you are concerned or have ongoing symptoms, please make an appointment with your GP or specialist.

Several factors may increase the risk of developing non-Hodgkin lymphoma. These include:¹

• Having a close relative with non-Hodgkin lymphoma
• A weakened immune system, which may occur due to autoimmune diseases (such as rheumatoid arthritis or coeliac disease) or medicines that suppress the immune system after an organ transplant
• Certain viral or bacterial infections, including hepatitis C, Epstein–Barr virus (EBV), human T-cell leukaemia/lymphoma virus (HTLV-1), human herpesvirus 8 (HHV-8), and Helicobacter pylori
• Breast implant-associated anaplastic large cell lymphoma (BIA-ALCL), a rare lymphoma linked to certain types of breast implants

B-cell lymphomas make up around 85% of all non-Hodgkin lymphomas. Common types include:¹

• Follicular lymphoma – usually slow-growing. Under the microscope, the cancer cells form circular patterns called follicles within lymph nodes.
• Diffuse large B-cell lymphoma (DLBCL)– a fast-growing lymphoma and the most common subtype in adults.
• Small lymphocytic lymphoma (SLL) – a slow-growing lymphoma that is closely related to chronic lymphocytic leukaemia (CLL).
• Mantle cell lymphoma – develops in the outer region (mantle zone) of lymph nodes. It may appear slow-growing but often behaves more aggressively.

Less common B-cell lymphomas include:1

• Burkitt lymphoma – typically fast-growing
• Marginal zone B-cell lymphoma – often slow-growing

T-cell lymphomas account for approximately 15% of non-Hodgkin lymphomas. Types include:¹

• Peripheral T-cell lymphoma – often presents with widespread, painless enlarged lymph nodes.
• Cutaneous T-cell lymphoma – primarily affects the skin and may begin as red, scaly patches or itchy raised bumps.
• Angioimmunoblastic T-cell lymphoma – starts in lymph nodes and may cause skin rashes and abnormal protein production.
• Anaplastic large cell lymphoma – a fast-growing lymphoma that can occur in lymph nodes or other parts of the body.

Lymphomas arising from natural killer (NK) cells are rare. They are typically aggressive and are often associated with Epstein–Barr virus infection.

The main type is:

• Extranodal NK/T-cell lymphoma, nasal type – most commonly affects the nasal area

Very rare related conditions include aggressive NK-cell leukaemia and certain chronic lymphoproliferative disorders of NK cells.

If your GP is concerned about your symptoms or risk factors, they will refer you to a specialist for further assessment.

Your doctor will usually begin with a physical examination to check for enlarged lymph nodes and other signs of illness. Following this a range of tests may then be performed to confirm a diagnosis.

Lymph node biopsy

A lymph node biopsy is the main test used to diagnose non-Hodgkin lymphoma.1 There are two main types:

• Excision biopsy –part or all of a lymph node is removed during a minor surgical procedure
• Core biopsy –a needle is inserted into a lymph node to remove a small sample of tissue. This may be used if surgical removal of the entire lymph node is difficult

Further tests

If a biopsy confirms non-Hodgkin lymphoma, additional tests may be needed to determine how far the lymphoma has spread (staging).¹ These may include:

• Blood tests – including a full blood count to measure red cells, white cells and platelets. Blood tests may also assess kidney and liver function.
• Imaging tests – such as:
  • Ultrasound (often used to guide a core biopsy)
  • Chest X-ray (to check for enlarged lymph nodes in the chest)
  • PET-CT scan of the chest, abdomen and pelvis (to assess the extent of disease)
• Bone marrow biopsy– a small sample of bone marrow is taken, usually from the pelvic bone, and examined under a microscope

If you are diagnosed with non-Hodgkin lymphoma, your doctor will explain the stage of your disease. Staging describes how far the lymphoma has spread in the body.

There are four stages:

• Stage 1 – lymphoma is limited to one lymph node region or a single area outside the lymph nodes
• Stage 2– lymphoma is in two or more lymph node regions on the same side of the diaphragm
• Stage 3 – lymphoma is found on both sides of the diaphragm
• Stage 4 – lymphoma has spread to one or more organs outside the lymphatic system

Stage 1 is the least advanced stage, and Stage 4 is the most advanced.¹

Doctors also assign a letter A or B:¹

• A – you do not have ‘B symptoms’ (unexplained fevers, drenching night sweats, or significant unintentional weight loss)
• B– you have one or more of these symptoms

Some treatments for lymphoma, particularly certain types of chemotherapy, can cause temporary or permanent infertility.1,2

Depending on your age and future plans, your doctor may refer you to a fertility specialist before treatment begins. Options may include:

• Semen cryopreservation (sperm freezing)
• Oocyte cryopreservation (egg freezing)
• In vitro fertilisation (IVF) to collect and store embryos

Early discussion is important, as fertility preservation usually needs to occur before treatment starts.

If you are diagnosed with a low-grade lymphoma, your treating doctor may recommend monitoring the cancer with regular check-ups rather than commencing treatment.¹

Chemotherapy is an approach to cancer therapy that involves the administration of medicine, usually orally or by injection, which is intended to kill cancer cells or minimise their growth and spread. Chemotherapy can be used in conjunction with other cancer treatments.³

Immunotherapy is a type of treatment that is intended to help your own immune system fight cancer. There are different types of immunotherapy for cancer that work in different ways.⁴

Radiation therapy may be offered in the early stages of cancer treatment, or after it has started to spread. There are different radiation therapy techniques which may be suitable for different cancer types at different stages.⁵

Surgery may be used to help treat lymphomas that have started in organs such as the spleen, thyroid or stomach, and that have not spread from these organs. It can sometimes also be used to treat certain types of skin lymphomas.⁶

This type of treatment is designed and aims to attack specific cancer cells. Targeted therapy medicines mimic antibodies that our bodies naturally produce that target and attach to proteins on cancer cells, making it easier for our immune system to find and destroy the cancer.¹

Steroids are chemicals naturally made in the body that can also be produced in a lab and then used as a medicine. Steroids may be used to increase the effect of chemotherapy, to seek to destroy lymphoma, and to help treat certain potential side effects.¹

A stem cell transplant may be recommended if your Non-Hodgkin Lymphoma comes back or doesn’t respond to other treatments.¹ For stem cell transplant your bone marrow has to be destroyed with a high dose chemotherapy treatment. This gets rid of the damaged or destroyed blood stem cells in the bone marrow, which are then replaced with healthy stem cells that can turn into bone marrow. These can be your own blood stem cells or from a donor.¹