Many people have no symptoms when first diagnosed with CLL, which is often discovered by chance during a routine blood test. Other people may have enlarged lymph nodes, splenomegaly or hepatomegaly on physical examination.
Watchful waiting (close monitoring with no treatment until signs or symptoms appear or change) is an option for some people with asymptomatic CLL. Others will need to start therapy relatively soon after diagnosis.
Symptoms
A type of blood cancer, CLL is a slow-growing disease that can be found at any age. It starts when abnormal lymphoid stem cells develop into cancerous B lymphocytes, or leukemia cells. Over time, these cancerous cells build up in the bone marrow and the bloodstream and crowd out healthy lymphocytes, red blood cells and platelets. This can lead to low levels of these blood cells, called anemia. It can also cause enlarged lymph nodes in the neck, underarms or groin.
Some people with CLL do not have any symptoms, which is why it’s important to get regular health check-ups. Your doctor will ask you about your general health and symptoms, such as swollen glands in the neck or armpits that feel firm and full, or fatigue. Then they’ll do blood tests and a physical examination to look for signs of CLL, such as abnormally high numbers of white blood cells or B lymphocytes in the blood.
The doctor will decide if you need treatment right away. They might use a test called absolute lymphocyte count doubling time, which measures how quickly the number of lymphocytes is growing. This can help them predict how fast the leukemia is progressing. They might also recommend a period of watch and wait, where you have regular check-ups but don’t receive treatment.
If your doctor recommends treatment, they may use a drug called ibrutinib. It targets proteins that help cancer cells grow and spread, but spares normal cells. It has fewer side effects than other drugs used to treat CLL. It’s usually given as pills. It might be given with other medications, such as chemotherapy or radiation therapy to shrink an enlarged spleen and improve blood cell counts. Surgery is rarely used to treat CLL, but it can be done if an enlarged spleen causes pain or other problems.
Your doctor might also suggest a clinical trial. These are research studies that test new ways to treat diseases, including CLL. You might be able to take part in one of these studies, depending on your health and where you live.
Diagnosis
A health care provider will do a physical exam and ask about your symptoms. They may also run tests on your blood and bone marrow to see how the cancer cells are growing. These include a complete blood count, a flow cytometry test to find out if the leukemia cells are B or T cell, and other tests that look at the genes and chromosomes in the cells. Your doctor will use these results to determine how well you respond to treatment.
For most people with CLL, the cancer grows very slowly and has no symptoms. This is called indolent CLL. For other people, the cancer cells grow faster and can cause symptoms. The symptoms depend on the stage of the disease and whether it spreads to the lymph nodes, liver or spleen.
CLL starts when stem cells that form blood cells start to grow abnormally and multiply out of control. The abnormal cells, which are called lymphocytes, crowd the bone marrow and interfere with the production of normal red blood cells, platelets and other blood cells. This causes the number of red blood cells and platelets in your body to fall below normal levels. It can also lead to enlarged lymph nodes (glands) that press on organs like the stomach and liver.
When lymphocyte counts increase, a health care provider can test them over time to see how rapidly the cells are growing. This is called lymphocyte doubling time or LDT. If the lymphocytes are growing quickly, you will probably need treatment right away. This is a good time to talk to your provider about joining a clinical trial.
Some people with CLL develop antibodies that attack their own red blood cells and platelets, causing immune-mediated anemia or thrombocytopenia. This increases the risk of infections and can lead to severe or life-threatening problems, such as pneumonia and sepsis. These problems can be managed with medication or treated with a bone marrow transplant. The risk of infection can also be reduced by keeping up with routine vaccinations, avoiding live vaccines and getting antibiotics when needed.
Treatment
The most common treatment for CLL is fludarabine. This is an anti cancer drug that targets lymphocytes. It is given through an IV, five days a week for four to six months. It is usually well tolerated with only fatigue as a side effect. Most people with CLL will be able to achieve remission with this therapy.
Unlike acute leukemias, which progress very quickly, chronic lymphocytic leukemia develops slowly over a period of years. It is often not discovered until a blood test is done for another reason. The disease can often be found in an advanced stage and is difficult to treat.
Most people with CLL are asymptomatic (have no symptoms). In most cases, doctors will recommend treatment only if there is severe fatigue interfering with daily activities or the lymphocyte count (the number of B cells in the blood) doubles in less than 12 months. Some people with this type of cancer are at high risk of converting to more aggressive forms of lymphoma, such as diffuse large B cell lymphoma or Hodgkin lymphoma.
Some doctors will treat CLL with a combination of chemotherapy and a stem cell transplant to try to get the disease under control as quickly as possible. Stem cell transplants use healthy, young cells from another person to help your bone marrow produce new immune system cells. These can be from your own body or from a donor.
Your doctor may also give you drugs that boost your immune system to attack cancer cells. These are called immunotherapy drugs and they can be given by mouth or intravenously. They work differently than chemo and can cause different side effects.
It is important to follow your treatment plan and take all medications as prescribed. It is also important to watch for infections, especially viral infections like shingles and influenza, because they can be more serious for people with lowered immunity.
Clinical trials are research studies that test whether a promising treatment can help people with a certain condition. Your doctor can talk to you about clinical trials and help you find one that is right for you.
Clinical trials
Clinical trials are research studies that offer patients the opportunity to try new treatments for a disease before they become available on the market. These research studies are carefully monitored by experts to ensure the participants are receiving safe treatment and not experiencing adverse side effects. Patients participating in a clinical trial can stop at any time for any personal or medical reason.
The EVOLVE CLL/SLL study is currently enrolling high-risk patients in the US. These are patients who have unmutated IGVH, 17p deletion, or other features associated with a higher risk of developing CLL. The study is randomizing these patients to immediate treatment with venetoclax/obinutuzumab or standard of care, which is watchful waiting for a period, then starting traditional therapy when symptoms develop. This is an exciting and innovative way to approach the management of high-risk CLL/SLL patients.
Researchers are also studying a variety of different chemotherapy drugs in combination with ibrutinib to see which combinations are most effective. For example, adding rituximab to fludarabine and cyclophosphamide has been shown to improve outcomes in CLL patients. The ibrutinib-rituximab combination is being studied further in the CAPTIVATE study.
Some studies are also looking at using a number of different genetic markers to predict whether the patient will respond to specific therapies, including ibrutinib. This information is helpful in tailoring the dose and schedule of a patient’s treatment regimen.
Lastly, several trials are investigating the impact of palliative care on patients with advanced-stage CLL. These studies are important, as current treatment options can cause a wide range of side effects that can significantly interfere with a patient’s quality of life.
It is important for patients to discuss their options with their healthcare provider, and to consider participation in a clinical trial. By doing so, patients are contributing to advancements in the treatment of CLL/SLL, and helping to shape future CLL cures. If a patient decides to participate in a clinical trial, it is critical that they understand the purpose of the study, what will happen during and after the clinical trial, all potential risks and benefits, and have an understanding of their privacy rights.
