Chronic Lymphocytic Leukaemia (CLL)

The reason that some people get CLL is not completely clear. However, there are some factors that are known to increase the chance of a person developing this disease called ‘risk factors’. Having a risk factor does not mean that you will definitely get the disease, but the more risk factors you have can make it more likely. Some risk factors for CLL are:

• Certain cultural groups like Ashkenazi Jews.
• Age and sex (CLL is more common in older people and men).
• Living on a farm or exposure to pesticides or herbicides.
• Reduced recreational sun exposure.
• Medical history of allergy to a body part.
• Family history of the disease.
• Exposure to Hepatitis C virus.
• Common infections.

Understanding what causes CLL will help researchers find more effective ways to treat it.

If your doctor thinks you might have CLL, they will ask questions about symptoms, examine a certain part of the body to see an increase in the size of certain parts of the body like liver, spleen and lymph nodes, refer you to a blood-cancer specialist, or run certain blood tests.

• Blood tests: A routine blood test called a complete blood count (CBC) can be used to show the increased number of white and red blood cells.
• Flow cytometry: Specific chemicals and dyes can identify special cell markers, which differentiate leukemia cells from the regular lymphocyte cells.
• Genomic testing: Leukemia cells can be observed for the presence of specific proteins, genetic changes and other leukemia specific factors.
• Imaging tests: A small amount of high energy particles (X-rays) can be used to take pictures of the inner body structures. Alternatively, a Computed tomography (CT or CAT scan) uses a computer to create a detailed, 3-dimensional image combining different angled pictures of the inside of the body.
• Bone marrow aspiration or biopsy: The liquid part (aspiration) or solid part (biopsy) of the bone marrow is sampled with a needle so it can be examined.

The stage of CLL describes where the cancer is and if it has spread into nearby tissues and/or around the body. This can help doctors decide how best to treat the disease. The staging system that is commonly used in Europe for CLL is called the Binet Staging System:

• Stage A – The person doesn’t show lower capacity of blood to carry oxygen to body parts (anaemia) or a lower platelet count in the blood (thrombocytopenia). Less than three organs or tissues that protect the body from damage by foreign materials (lymphoid) are involved.
• Stage B – The person doesn’t show anaemia nor thrombocytopenia. The CLL shows three or more areas of lymphoid involvement.
• Stage C – The person shows anaemia and/or thrombocytopenia irrespective of the number of areas of lymphoid involvement.

CLL and Small lymphocytic leukemia (SLL) are basically the same disease, the only difference being the location of the cancer cells. In CLL, most of the cancer cells are located in the bloodstream and the bone marrow (although the lymph nodes and spleen are often involved), while in SLL the cancer cells are located mostly in the lymph nodes.

At diagnosis, about 10% of people with CLL report a family history of the condition, or of a similar lymphocyte disorder. Having a family history of CLL can increase a person’s risk for having it themselves. It is also commonly seen that people who have low ability to resist infections are at an increased risk of developing CLL.

In the majority (68%) of people with a family history of CLL, the disease is caused by a change (known as a ‘mutation’) in the genes which form part of the structure of antibodies.

Treatment of CLL depends on the subtype and stage of the disease. Other things that are thought about when deciding on treatment are age, overall health and overall physical condition.

Treatments for CLL include:

• Inhibitory cell targeted treatments: Outstanding progress has been seen after the introduction of agents targeting specific cells. These agents successfully interfere with the cancer developing process in the body or work on regaining balance on the body’s unwanted cell elimination process.
• Chemotherapy: Chemotherapy plays a marginal role in treating CLL. An oral tablet is taken three to five days at the start of each treatment cycle and is usually done at home. Other anticancer medications can be given directly into a vein, which requires an overnight stay at the hospital. Alternatively, several other anti-cancer medicines could also be used along with medicines which reduce inflammation (steroids).
• Stem cell or bone marrow transplants: Stem cells or bone marrow transplants are sometimes used to try to get rid of CLL completely or control it for longer periods. Stem cells are produced in the bone marrow and can turn to different types of blood cells, including white blood cells. In this procedure, either stem cells or bone marrow cells are taken from a healthy person and given to a person with CLL, replacing their unhealthy cells with healthy ones.
• Radiotherapy: This treatment is usually given in the hospital, where high energy radiation waves are used to kill cancer cells. This could include external radiation, implants of radioactive material, radioisotope injections, capsules or drinks, or intrabeam therapy.
• Other treatments that support the problems caused by CLL like frequent infections include surgery, antibiotics, antifungals, transferring donated blood to the person with CLL (blood transfusions), treatments that boost antibody levels (immunoglobulin replacement therapy) and medicines to increase the number of healthy white blood cells.

Clinical trials (which can also be called ‘research studies’) are designed to look at how safe experimental drugs or procedures (such as new types of surgery) are and how well they work. Researchers are working hard to learn more about CLL and new therapy options are available.

As researchers learn more about CLL, clinical trials will be set up to look at potential new treatments.

If you would like to know more about Roche sponsored clinical trials or are interested in taking part in a clinical trial, speak to your doctor or visit the Roche ForPatients clinical trials page Roche ForPatients clinical trials page.