Chronic lymphocytic leukaemia (CLL)

CLL can affect organs like the spleen and liver.

Your spleen is an organ in the upper left side of your abdomen, under your ribs. It helps filter your blood and store blood cells. In CLL, the spleen can become enlarged. This is called splenomegaly. CLL can also cause the liver to become enlarged.

An enlarged spleen or liver may cause swelling or a feeling of fullness in your abdomen. You may also feel discomfort or pain. An enlarged spleen can make you feel full quickly when eating.

If CLL has spread to your lymph nodes you may feel small, hard lumps in your armpits, on either side of your neck, near your collarbone and/or in your groin.

They usually won’t be painful, although they can cause other symptoms as they grow. For example, if you have an enlarged lymph node in your neck, you may have a cough.

There are internal lymph nodes too, in your chest and abdomen. They might swell but can only be seen with scans.

• Fewer than 3 areas of lymph tissue are enlarged.
• Normal red blood cell and platelet levels.
• There are usually no symptoms.
• Considered low risk disease.

• At least 3 areas of lymph tissue are enlarged.
• Normal red blood cell and platelet levels.
• Symptoms include being tired with low energy.
• Considered intermediate risk disease.

• Multiple areas of lymph tissue may be enlarged.
• Lower than normal red blood cell and platelet levels.
• Symptoms can include tiredness, bruising or bleeding, frequent infections, weight loss and night sweats.
• Considered high risk disease.

Some people have a blood condition called monoclonal B-lymphocytosis (MBL). MBL isn’t leukaemia, it is sometimes called pre-CLL. In MBL, the B cells don’t work properly. There are 3 types of MBL:

• low count MBL
• CLL/SLL-type MBL
• non-CLL/SLL-type MBL.

Each year, 1% to 2% of people with CLL/SLL-type MBL will develop CLL.

People with MBL will have regular blood tests and follow up with their general practitioner (GP), but do not need treatment.

Rarely CLL progresses to another type of lymphoma. In this type of lymphoma you will have a higher than normal number of prolymphocytes, a type of white blood cell.

It can be difficult to treat and has a poorer prognosis than CLL.

Richter transformation is a rare complication of CLL. It is when CLL transforms into a more aggressive form of lymphoma, usually diffuse large B-cell lymphoma (DLBCL) or Hodgkin lymphoma (HL).

This can affect about 7% of people with CLL.

A biopsy of a lymph node or a lump can help diagnose CLL. A biopsy is a procedure that involves taking a sample of tissue from a swollen lymph node or a lump and looking at it under the microscope.

A bone marrow biopsy is a test that can help diagnose CLL. A bone marrow biopsy uses a needle to take a sample of bone marrow, usually from the back of the hip. A small amount of liquid bone marrow is removed and sent to the laboratory for testing. Often, a tiny piece of the bone itself is also taken to be examined.

Other tests you may have include:

• medical history and physical exam
• blood tests – full blood count (FBC), kidney and liver function, electrolytes
• genetic tests
• computed tomography (CT) scan
• positron emission tomography (PET) scan
• magnetic resonance imaging (MRI).

These tests help your treatment team work out your stage and prognosis.

Active monitoring involves regular blood tests and health checks to see if the disease has progressed. No treatment is needed unless you have signs and symptoms which show the chronic lymphocytic leukaemia (CLL) is progressing.

Studies show that active monitoring offers no disadvantages compared to early treatment for low risk CLL. In some cases, CLL can be managed with this approach for years before it progresses.

Supportive care prevents and treats symptoms and side effects. It can include blood transfusions and medications to improve blood counts, as well as emotional, social, spiritual and informational support.

The goal is to improve symptoms of your CLL, but it doesn’t treat the disease itself. People with stage B and C (intermediate-high risk) CLL may have supportive care alongside treatment.

Cancer growth inhibitors are a type of targeted therapy. They block signals to cancer cells to stop them from growing or surviving. There are a few types of cancer growth inhibitors used to treat CLL.

Venetoclax is a B-cell leukaemia/lymphoma-2 (BCL2) inhibitor. BCL2 is a protein made by some cancer cells. BCL2 inhibitors block this protein, causing the cancer cells to die. They are taken as tablets and are usually given in combination with immunotherapy.

Ibrutinib, acalabrutinib and zanubrutinib are Bruton’s tyrosine kinase inhibitors (BTKis). Bruton’s tyrosine kinase (BTK) is a protein that helps B lymphocytes grow. Because CLL is a cancer of B lymphocytes, the BTKis’ block this protein and the cancer cells die. They are taken as tablets.

Immunotherapy is a type of biological therapy. It uses the immune system to recognise cancer cells and destroy them. Your treatment team will give this to you via a drip into a vein (intravenous).

Immunotherapy works by:

• stimulating the immune system to find and attack cancer cells
• removing barriers that are stopping the immune system killing cancer cells.

Obinutuzumab and Rituximab are monoclonal antibodies used to treat CLL. They damage cancer cells and cause them to die by attaching to a protein called CD20 on the blood cancer cell. They are given as intravenous infusions, usually in combination with a cancer growth inhibitor or chemotherapy. 

Chemotherapy is a less common treatment for CLL. Chemotherapy kills cancer cells and stops them from growing. It also damages healthy cells and causes side effects. There is information on chemotherapy side effects and how to manage them on our chemotherapy webpage.

Chemotherapy drugs for CLL are often given in combination with immunotherapy drugs. Treatment plans for CLL are shortened to letters like FCR. The letters represent the name of the medicines in that plan. Your haematologist will provide you with information on your plan, including when and how often you will have it. 

There are several treatment plans for CLL. They can be found on the Cancer Institute NSW website.

Radiation therapy (also called radiotherapy) is sometimes used to treat CLL. It destroys cancer cells:

• using high energy x-rays
• in the local area it treats.

CAR T-cell therapy is a type of treatment that uses your own immune cells to fight cancer.

Your T cells are collected from your blood and changed in a laboratory so they can find and attack your blood cancer cells. After you have chemotherapy to prepare your body, the CAR T-cells are returned to you through a drip into a vein.

Your treatment team may recommend CAR T-cell therapy if your CLL transforms into an aggressive form of lymphoma, like diffuse large B-cell lymphoma (DLBCL). Not everyone is eligible for CAR T-cell therapy. Your treatment team will discuss whether this treatment is suitable for you.

A bone marrow or blood stem cell transplant is a treatment that restores stem cells after high-dose chemotherapy. Your treatment team might suggest a transplant if your CLL progresses or transforms into a more aggressive type of cancer. There are 2 types of transplants:

• An autologous stem cell transplant  is when your own stem cells are collected and then given back to you after you receive high dose chemotherapy.
• An allogeneic stem cell transplant is when you receive stem cells from a donor, often a blood relative.

It is more common to receive an allogeneic transplant for CLL.

Clinical trials may be available for some types of CLL. They often compare new or combination therapies with current treatments, including side effects.

Many are randomised, meaning some patients receive the new treatment, and others receive standard care. Trials help improve future treatments and may offer access to costly new therapies not on the Pharmaceutical Benefits Scheme (PBS) in Australia.

If you are considering a clinical trial, your doctor will explain:

• the possible risks and benefits
• how it compares to standard treatment
• what is involved.

You will need to give informed consent before taking part.