CLL is a cancer of the mature lymphocytes, a type of white blood cells. These cells can be found in the blood, bone marrow, lymph nodes or spleen. With CLL, a B lymphocyte (type of white blood cell) loses its ability to live and die normally. As a result, it multiplies into many CLL cells. The CLL cells grow and survive better than normal cells. As CLL cells increase in number, they start to take up space in the bone marrow and crowd out the normal red cells and platelets. CLL doesn’t completely interfere with the development of mature red cells, other white cells and platelets. However, your number of healthy blood cells is usually lower than normal.
Causes and Risk Factors
We don’t know what triggers the development of leukemia cells. For most people who have CLL, there are no obvious reasons why they developed the disease. Researchers have concluded that there’s no way to prevent CLL. It is not common, but in some families, more than one blood relative has CLL or another blood cancer.
Signs and Symptoms
Some people with CLL have no symptoms. They find out they have CLL when a routine blood test shows changes to their blood. CLL symptoms often develop slowly. People with CLL may have little or no change in their health for many years.
You may experience these signs and symptoms:
Fatigue, paleness, and shortness of breath
When your red cell count is low (anemia).
When you are eating less and/or using more energy, you may lose weight quickly.
Bleeding for a long time from minor cuts
When your platelet count is low (thrombocytopenia) your body heals more slowly.
Higher risk of infection
CLL cells may not function properly, particularly in the production of antibodies and fighting viral infections. As a result, your immune system can’t effectively guard against infection.
Swollen lymph nodes and spleen
When CLL cells accumulate, this can be the result.
Cancer screening and skin checks are important. Someone with CLL has a higher risk of other cancers, especially skin cancers. The immune system, whose normal job is to remove abnormal cells, may not be able to recognize or remove these cells. Because these symptoms can also occur with other diseases, it is important to have your doctor figure out the cause of your symptoms. They will usually start by interviewing you, examining you and then testing your blood if necessary.
It is important to be properly diagnosed using blood test results. These tests include:
Complete Blood Counts
This test measures the number of red cells, white cells and platelets in your blood. A person with CLL usually has a high number of lymphocytes, a type of white blood cell that fights infection. You may also have low red blood cell and platelet counts.
This test classifies cells in a blood sample according to cell surface proteins, which can help determine whether you have CLL.
Other lab tests for CLL include:
Immunoglobulins are proteins that help your body fight infection. People with CLL may have low levels of immunoglobulins. A low immunoglobulin level may be the cause of repeated infections. Your doctor may check your immuno globulin level to see if there is a drop in immune function. If you meet the criteria for antibody replacement, you might need an injection of antibodies to maintain immune function or prevent infections.
Bone Marrow Aspiration and Biopsy
These tests examine your bone marrow cells for abnormalities. For a bone marrow aspiration, a sample of bone marrow is collected from the hip bone. For a bone marrow biopsy, a sample of solid bone is collected that contains marrow. These tests are typically not needed for a diagnosis. They are recommended before treatment begins so that your medical team can watch for changes after treatment begins.
Fluorescence in situ hybridization (FISH)
Every cell in the body has chromosomes that carry genes. Genes give the instructions that tell the cell what to do. This test is used to see if there are changes to the chromosomes of the CLL cells. More than half of people with CLL have cells with chromosome changes. The FISH test uses blood or marrow cells to help your doctor determine which treatment is best for you.
Gene sequencing will determine whether you have certain changes to your genes. Your treatment plan will take these into consideration.
Treatment options for CLL can vary greatly. If your CLL is slowgrowing, you may not need immediate treatment. If your CLL is growing at a faster rate, you may need treatment sooner.
The slower-growing form has an increased number of lymphocytes but a normal or slightly below the normal level of red cells, platelets and neutrophils (another type of white cell) in the blood. This form can remain stable for years.
The faster-growing form has too many CLL cells in the blood that block normal red blood cell and platelet production. As a result, you have lower counts of red cells and platelets.
The goals of CLL treatment are to slow the growth of your CLL cells and help you enter periods of remission when you can maintain your quality of life with no signs of CLL. Your treatment will also help you feel better if you have infections, fatigue or other symptoms.
It is important to talk to your doctor about the results and goals of your treatment. With CLL, responses to treatment vary a lot and depend on many factors including:
• Stage of CLL – this is based on risk factors including swollen lymph nodes, liver, or spleen, low red blood cell or platelet count
• Physical exam and lab test results • Your overall health
• Your age
• The molecular profile of your CLL
There is currently no cure for CLL, but treatments can help you manage your disease.
Your treatment plan may include:
Watch and Wait (Active Observation or Active Surveillance)
If you have slowgrowing CLL and no symptoms, your doctor may decide to not start treatment. This is known as a watch and wait period. A watch and wait period helps to avoid the side effects of treatments on your body when you have not met international criteria set by worldwide experts in CLL (iWCLL). You will have regular physical exams and lab tests. This helps your doctor to detect changes and determine the best way to treat your CLL. Treatment will begin if you have symptoms or lab results showing that your CLL meets iWCLL criteria to be treated.
Chemotherapy is treatment with drugs that kill or damage cancer cells. The drugs are given orally or through an intravenous (IV). Often, two or more drugs are used together to make them more effective depending on how well a person tolerates them.
These treatments have specific targets on or within cancer cells and side effects may be different from those of chemo therapy. Targeted therapies are given in pill form and are becoming more common especially in highrisk patients.
Monoclonal Antibody Therapies
These labmade antibodies attach to specific markers on the surface of CLL cells and destroy them. Monoclonal antibody therapy is most often given in combination with other treatment options. The side effects of monoclonal antibody therapies are specific to their type and how they are given. These treatments can be given through an IV or injected under the skin.
This treatment kills cancer cells using highenergy rays such as xrays. If you have swollen lymph nodes or organs, radiation therapy may be part of your treatment plan. However, it is not common for CLL to be treated with radiation.
Treatment for Relapsed or Refractory CLL
CLL is incurable and is expected to relapse. CLL is relapsed if it first responds to therapy, but eventually stops responding. Refractory CLL does not enter remission.
With treatment or progression, you can gain additional chromosomal changes or genetic mutations. If you have refractory CLL, you will be tested further for these. You will receive more testing if you have relapsed CLL and meet the criteria for treatment. Your doctor will use the results to decide on your next therapy option.
People who are treated for relapsed or refractory CLL often have years of remission after more treatment. New drug therapies can be used to treat relapsed or refractory CLL. There are also clinical trial options.
Side Effects of Treatment
CLL therapies kill CLL cells but they also affect healthy cells. People react to treatments in different ways. Side effects can vary in duration and severity. You will be monitored throughout treatment, as some side effects appear early in treatment and others later.
Some general side effects of CLL treatment include: aches, diarrhea, constipation, fatigue, infections, low blood pressure, low platelet count, low red blood cell count, low white blood cell count, mouth sores, upset stomach and vomiting. Some of the newer treatments have specific side effects that your health care team will discuss with you.
Minimal Residual Disease
After treatment, some people with CLL have a very low level of remaining CLL cells. The remaining CLL cells are called minimal residual disease (MRD) and are not detected by regular bone marrow or blood tests. MRD is detected by more sensitive diagnostic tests. These tests may help your doctor to determine whether you need further treatment but is not routinely used in Canada.
Medical followup is important after treatment for CLL. You may require blood tests, bone marrow tests, or molecular tests to determine if you need treatment. Your medical team should provide you with a care plan listing the frequency of followup visits and the tests you will have at those visits.
It is common to have longterm side effects that last for months or years after treatment ends. Fatigue is a common example. Late effects are medical problems that do not show up until years after treatment ends. You will need to see the doctor for followup care for potential early detection of heart disease, secondary cancers, fertility problems, thyroid problems, problems concentrating, and chronic fatigue.
You should seek medical help if you feel “down” or “blue” or don’t want to do anything and your mood does not improve over time. These could be signs of depression. Depression is an illness and should be treated even when you are also being treated for CLL. Treatment for depression has benefits for people living with cancer, and you are not alone.
LLSC gratefully acknowledges Dr. Versha Banerji, MD, FRCPC and Dr. Spencer Gibson, PhD, for their important contribution to the content of this publication.
For more information, don’t hesitate to contact us: 1-833-222-4884 • firstname.lastname@example.org • llscanada.org