Leukemias are cancers of lymphocytes, the cells that make up part of your immune system and help your body fight infections. Lymphocytes are also known as white blood cells and include NK (natural killer) cells, B-cells, and T-cells. CLL is a cancer of the B-cells, which are cells that produce antibodies that attach to bacteria, viruses, and other invading organisms in the body and target them for destruction by the immune system. Although CLL means you will produce a large number of immune cells, these cells do not function like normal cells and patients with CLL are more likely to get sick. Since CLL is a cancer of blood cells, it can spread anywhere in the body, but the cancer cells are usually found in the blood, bone marrow, and lymph nodes.

Cancer occurs when cells of the body begin to grow and divide out of control. Although CLL may be a fast- or slow-growing cancer, it usually grows slowly and may not be detected for years. Over time, CLL cells will crowd out healthy normal cells in the bone marrow. This makes it difficult to produce red blood cells and platelets. Once this happens, you may begin to experience symptoms of CLL, such as tiredness and fatigue, weight loss, and frequent illnesses. CLL may also spread to your lymph nodes, liver and spleen, and cause these organs to enlarge.

National Comprehensive Cancer Network. NCCN Guidelines for Patients. Chronic Lymphocytic Leukemia. https://www.nccn.org/patients/guidelines/cll/12/. Accessed March 6, 2019.

Blood tests may be performed to help diagnose CLL, determine your prognosis (outlook), or determine the stage of your cancer. Some of the tests and the meaning of the results are included below:

• FISH test is used to determine if the CLL cells have any abnormal chromosomes or genes. Missing or deleted parts of chromosome 17 or 11 are signs of a poor prognosis. A good prognosis is associated with deleted parts of chromosome 13, as long as it is the only chromosomal abnormality found. Trisomy 12, in which there is an extra copy of chromosome 12, is not linked to a better or worse prognosis.
• Complex karyotype is linked to a worse prognosis. A karyotype is a picture of all the chromosomes in a cell. A complex karyotype occurs when there are 3 or more unrelated abnormalities in the chromosomes of more than one cell.
• IGHV refers to the gene that produces part of the structure of an antibody, an infection-fighting protein created by a B-cell. The IGHV gene may or may not be mutated in CLL cells. Patients with a mutated IGHV gene have a better prognosis than patients with an unmutated version of the gene.
• TP53 refers to a gene that controls whether a cell with damaged DNA is repaired or destroyed. Damaged cells may become cancerous if they are allowed to grow and divide. Mutations of TP53 lead to an increased risk of cancer growth, resistance to chemotherapy, and poor prognosis. TP53 mutations are often seen in patients with deletions of part of chromosome 17. Certain new targeted therapies, such as ibrutinib and venetoclax, are effective in treating CLL with TP53 mutations.

National Comprehensive Cancer Network. NCCN Guidelines for Patients. Chronic Lymphocytic Leukemia. https://www.nccn.org/patients/guidelines/cll/18/. Accessed March 6, 2019.

Not all patients with CLL need to begin treatment right away. The decision to begin treatment is based on symptoms of CLL, test results, and the stage of the cancer. The cancer stage is a rating determined by the characteristics of the cancer that suggests your likely prognosis, or outcome. One system that is used to determine the stage of CLL is the Rai system. There are 5 stages:

• Stage 0: normal test results except for a high number of lymphocytes in the blood. The likelihood of the cancer getting worse is low. Treatment is usually not needed and observation is recommended.
• Stage I: high number of lymphocytes in the blood and enlarged lymph nodes. There is an intermediate risk of the cancer getting worse. Treatment is usually not needed unless the patient has symptoms. Most patients with stage I CLL are monitored and observed for disease progression.
• Stage II: the patient has an enlarged liver, spleen, or both. There is an intermediate risk of the cancer getting worse. Treatment is usually not needed unless the patient has symptoms. Most patients with stage II CLL are monitored and observed for disease progression.
• Stage III: the patient has a low hemoglobin level. The likelihood of the cancer getting worse is high. Most patients with stage III CLL will need to be treated, especially if blood cell counts continue to fall.
• Stage IV: the patient has a low platelet count. The likelihood of the cancer getting worse is high. Most patients with stage IV CLL will need to be treated, especially if blood cell counts continue to fall.

National Comprehensive Cancer Network. NCCN Guidelines for Patients. Chronic Lymphocytic Leukemia. https://www.nccn.org/patients/guidelines/cll/18/. Accessed March 6, 2019.

CLL is a slow-growing, chronic illness and many patients will not need to be treated for many years, or even decades, after their diagnosis. In fact, approximately 30% of CLL patients will never need treatment. Some studies have shown that early treatment of CLL does not improve a patient’s chance of survival any more than waiting and watching for disease progression. All cancer treatments have side effects and it is best to avoid therapy for CLL until the disease progresses or gets worse.

CLL Society. https://cllsociety.org/. Accessed March 22, 2019.

Treatment for CLL should be a shared medical decision between you and your doctor. If you begin to experience signs or symptoms of CLL, such as drenching night sweats, unexplained fever or weight loss, and severe fatigue, you should let your doctor know as these may be signs that your CLL is progressing or getting worse. Your doctor will also monitor for changes in your lab results, such as a rapid increase in lymphocyte count, or increases in the size of your lymph nodes or spleen.

CLL Society. https://cllsociety.org/. Accessed March 22, 2019.

Talking often with your healthcare team and asking questions are important for making informed decisions about your health. There is no one-size-fits-all approach to treating CLL because the disease looks different from one individual to another. Some people may not need treatment while others do. Some people may show signs and symptoms of CLL while other people do not. It is vital that you receive personalized information about your cancer and health. Many people feel anxious about questioning their doctor or worried that they may not understand medical terms. The following strategies may help you talk to your doctor and get the information you need:

• Take someone with you to doctor visits who can take notes, listen, and ask questions.
• Ask as many questions as you need to. Write down your questions and bring them with you to appointments.
• Remember that all of your questions are important. There is no such thing as a silly question when it comes to understanding your health.
• Ask the doctor to explain any medical terms you don’t understand and write down any terms you don’t know.
• Repeat back the information the doctor gives you in your own words to make sure you understand it.

The following is a list of questions about CLL that you may want to ask your doctor. Remember to write down any additional questions you have about your own health and bring them with you to your appointment.

• Is this a fast- or slow-growing leukemia?
• What were the results of my genetics test? How do the results impact my prognosis and treatment?
• Will I be treated immediately for my cancer or will you monitor my cancer over time for progression?
• What symptoms indicate that my cancer is getting worse?
• How do my age, health, and other factors affect my treatment options?
• What are my treatment options? What are the benefits and risks of each option?
• Will I need any vaccinations because of my condition? Will I not be able to have certain vaccinations?
• What can be done to relieve or prevent the side effects of treatment?

For a list of additional questions, please see the references below.

National Comprehensive Cancer Network. NCCN Guidelines for Patients. Chronic Lymphocytic Leukemia. https://www.nccn.org/patients/guidelines/cll/18/. Accessed March 6, 2019.

American Cancer Society. Questions to Ask Your Doctor When You Have Cancer. https://www.cancer.org/treatment/finding-and-paying-for-treatment/choosing-your-treatment-team/questions-to-ask-your-doctor.html. Accessed March 22, 2019.

Tumor lysis syndrome (TLS) is a life-threatening emergency that may occur after treatment for a fast-growing cancer, such as leukemia. TLS is caused by the rapid death of cancer cells after treatment. When the tumor cells die, they suddenly release their contents into the blood, which can potentially damage organs such as the kidneys, liver, heart and nervous system. If you are at high risk of TLS, you will receive your cancer therapy in the hospital so that you can be monitored for signs of TLS.

In order to prevent TLS, you will receive intravenous (IV) hydration as well as medications that lower the level of uric acid in the blood, such as allopurinol or rasburicase.

Risk factors for TLS include high white blood cell counts, high uric acid levels, kidney problems, dehydration, or late-stage cancers.

Let your doctor know immediately if you experience any signs or symptoms of TLS, including nausea and vomiting, diarrhea, swelling, shortness of breath, irregular heartbeat, cloudy or bloody urine, decrease in urine amount, back pain behind the lower ribs, weakness or low energy, seizures, muscle spasms or cramps, or pain in the joints.

Cancer.net. When to Call the Doctor During Cancer Treatment. https://www.cancer.net/navigating-cancer-care/how-cancer-treated/when-call-doctor-during-cancer-treatment. Accessed March 22, 2019.

Support groups can be an important resource for people diagnosed with CLL. They provide a safe space to discuss your difficulties, feelings, and concerns with others who understand how you feel. No one support group is right for everyone and it is important that you find one that makes you feel comfortable. Some groups are specifically focused on CLL, while others may include people with any type of cancer. They also vary in format, from online or telephone support groups to in-person meetings. Some may even involve social workers to help patients cope with issues. You can find support groups in your area through an online search or by asking your doctor or the staff of a local hospital. Use the resources below to begin your search:

CancerCare. Blood Cancers Patient Support Group. https://www.cancercare.org/support_groups?41-blood_cancers_patient_support_group

Leukemia and Lymphoma Society. Support Groups. https://www.lls.org/support/support-groups

Cancer Support Community. Find Support. https://www.cancersupportcommunity.org/find-support

Cancer.net. Finding Support and Information. https://www.cancer.net/coping-with-cancer/finding-support-and-information

American Cancer Society. Support Programs and Services. https://www.cancer.org/treatment/support-programs-and-services.html