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Understanding CLL
Incidence
Causes and Risk Factors
Symptoms and Signs
Diagnosis
Goals of CLL Therapy
Treatment Planning and Staging
CLL Treatment
Clinical Trials
Treatment Response and Follow-Up
Talking to the CLL Specialist
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Chronic lymphocytic leukemia (CLL) is one of four main types of leukemia. About 15,490 new cases of CLL will be diagnosed in 2009. It is estimated that 85,710 people in the United States are living with or are in remission from CLL. Many people with CLL live good-quality lives for years with medical care. There are a number of treatments for CLL. In recent years new therapies have been approved and other possible new treatments are being studied in clinical trials. Progress toward a cure is under way. CLL starts with a change (mutation) to the DNA of a single cell called a lymphocyte. In 95 percent of people with CLL, the change occurs in a B lymphocyte. In the other 5 percent, the cell that transforms from normal to leukemic has the features of a T lymphocyte or an NK cell. B-cells, T-cells and NK-cells are types of lymphocytes. The diagram below illustrates the process of lymphocyte development. 
Click here to view a larger version of this diagram.
Over time, the CLL cells multiply and replace normal lymphocytes in the marrow and lymph nodes. The high number of CLL cells in the marrow may crowd out normal blood-forming cells, and CLL cells are not able to fight infection like normal lymphocytes do. back to top CLL is more common in people who are 60 years and older than in younger adults. The number of people with CLL starts to increase after age 50. A small number of people are diagnosed with CLL in their 30s and 40s. Children do not get CLL. back to top
Doctors do not know what causes the cell-change that leads to CLL. There is no way to prevent CLL. You can't catch CLL from someone else.
CLL has generally not been associated with any environmental or external factors. However, the Institute of Medicine of the National Academy of Sciences issued a report, which concluded that there was "sufficient evidence of an association" between herbicides used in Vietnam and CLL. For more information, visit the US Department of Veterans Affairs Web site or call (800) 827-1000. In certain families, more than one blood relative has CLL. However, this is not common. Doctors are studying why some families have a higher rate of CLL. back to top Symptoms and SignsCLL signs and symptoms usually develop slowly. Some people with CLL do not have any symptoms. These people may find out they have CLL after a regular medical checkup shows certain changes in the blood. Early in the course of the disease, CLL often has little effect on a person's well-being. Many of the signs and symptoms of CLL are more likely to be caused by other illnesses. Specific blood tests and bone marrow tests are needed to make a diagnosis. Some signs and symptoms of CLL include Tiring more easily. People may have less energy due to fewer healthy red cells and more CLL cells.
Shortness of breath during normal activities. This is due to fewer healthy red cells and more CLL cells.
Swollen lymph nodes or spleen. High numbers of CLL cells can gather in the lymph nodes or spleen as the number of CLL cells grows.
Infections. People with a very high number of CLL cells building up in the marrow may have repeated infections of the skin or other parts of the body. This is because CLL cells cannot fight infection as well as healthy lymphocytes.
Weight Loss. Some people with CLL lose weight because they eat less and/or because they are using more energy.
Some patients may also have other symptoms, such as aches, fever or night sweats. back to top The diagnosis of CLL is usually made from blood and bone marrow tests. Blood Tests The testing for CLL includes blood cell counts and a blood cell examination. - Blood cell counts. A person with CLL will have a high lymphocyte count. He or she may also have a low red cell count and a low platelet count.
- Blood cell examination. The CLL cells are usually examined with an instrument called a "flow cytometer." The test is called flow cytometry or immunophenotyping. The test is done to find out if CLL is the reason for the high lymphocyte count. Flow cytometry also shows if the CLL is B-cell CLL or T-Cell CLL. B-cell CLL is most common.
- Immunoglobulin Test. Doctors check the immunoglobulin level in the blood. Immunoglobulins are proteins that help the body fight infection. People with CLL may have low levels of immunoglobulins. A low immunoglobulin level may be the cause of repeated infections.
Bone Marrow Tests and Cytogenetic Tests Bone marrow tests are not usually needed to make a CLL diagnosis. But it's often helpful to have a bone marrow aspiration and a bone marrow biopsy before treatment begins. The results of these tests serve as a baseline that is used later on to assess the effects of treatment. FISH or fluorescence in situ hybridization is a test used to see if there are changes to the chromosomes of the CLL cells. About half of the people with CLL have CLL cells with chromosome changes. FISH may give doctors information about which patients need more medical follow-up. FISH can be done with a sample of cells from blood or marrow.
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It is important to get treatment in a center where doctors are experienced in the care of patients with CLL.
The goals of CLL treatment are to - Slow the growth of CLL cells
- Provide long periods of remission (when there are no signs of CLL and/or people feel well enough to carry on their day-to-day activities)
- Help people to feel better if they have infections, fatigue or other symptoms.
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The treatment plan for a person with CLL depends on the
- Stage of the CLL (low, intermediate or high risk)
- Physical exam and lab test results
- Person's overall health
- Person's age (for some treatments).
CLL Staging Many doctors use a system called staging to help plan treatment for people with CLL. Many doctors use the "Rai staging system," which defines a person's risk as follows: Low-Risk CLL - High lymphocyte count in the blood and the marrow.
Intermediate-Risk CLL - High lymphocyte count in the blood and the marrow
- Enlarged (swollen) lymph nodes
or - High lymphocyte count in the blood and the marrow
- Enlarged (swollen) lymph nodes, liver or spleen.
High-Risk CLL - High lymphocyte count in the blood and the marrow
- Anemia (low red cell count)
or - High lymphocyte count in the blood and the marrow
- A low platelet count.
Other lab test results may show signs of faster-growing disease (higher-risk CLL). This means the person needs closer follow-up with the doctor. Blood Lymphocyte Doubling Time - a lymphocyte number that doubles in one year means that the person needs closer follow-up. Beta 2-Microglobulin (B2M) - B2M is a protein on CLL cells. A higher level of B2M may mean there are more CLL cells. back to top Watch and Wait The watch and wait approach means that a doctor observes a person's condition with physical exams and lab tests. The doctor does not treat the person with drugs or other therapies during the watch and wait period. Some patients with CLL may think that they should have treatment right away. But for people with low-risk (slow-growing) disease and no symptoms, it is best not to start treatment. The watch and wait approach allows the patient to avoid side effects until treatment is needed. Patients in watch and wait need follow-up visits with the doctor. At each office visit, the doctor will check for any health changes. The results of exams and lab tests over time will help the doctor advise the patient about when to start treatment and the type of treatment to have. A doctor may advise the patient to begin treatment if one or more of these signs develop: The number of CLL cells is much higher than it was The number of normal cells is much lower than it was The lymph nodes have become larger The spleen has become larger
A patient with any or all of these signs may start to feel too tired for normal daily activities. Drug Treatment People who have intermediate- and higher-risk (faster-growing) CLL are usually treated with combination chemotherapy and/or monoclonal antibody therapy. Chemotherapy is treatment with drugs that kill or damage cancer cells. Some drugs are given by mouth. Other drugs are given through a vein by placing a small needle in the arm (called an IV). Two or more drugs are often used together. The FDA-approved drugs fludarabine (Fludara®), cyclophosphamide (Cytoxan®), bendamustine (Treanda®) and other standard chemotherapies are used to treat people with CLL. Monoclonal antibody therapies for CLL are immune proteins that are made in the lab. They aim for a specific target on the surface of the CLL cells. The antibody attaches to the cell and then the cell dies. This type of therapy is given through a vein by placing a small needle in the arm (called an IV). Monoclonal antibody therapies do cause some side effects. In general, the side effects are milder than the side effects of chemotherapy. Rituximab (Rituxan®) and alemtuzumab (Campath®) are two of the monoclonal antibody therapies used to treat people with CLL. Rituxan is being studied in clinical trials and is used in combination with chemotherapy. Other new monoclonal antibodies are being studied in clinical trials to treat people with CLL. Two or more drugs are often combined for CLL treatment. Fludara is often combined with other drugs to treat people with CLL who need drug therapy. For example: Treanda combined with other drugs is being studied in clinical trials to treat people with CLL. For example: Supportive Care Supportive care is given to prevent or treat CLL symptoms and/or treatment side effects. Supportive care for CLL may include Antibiotics used to treat infections that are caused by bacteria or fungi. Infections are a risk for CLL patients. The disease and treatment lower the number of infection-fighting white cells in the blood. Patients with repeated infections may also get injections of immunoglobulin. Blood transfusions or red cell growth factors used to increase the red cell count. Anemia (low numbers of red cells) is a common side effect of chemotherapy. Examples of red cell growth factors are Aranesp® (darbepoetin alfa) and Procrit® (epoetin alfa). Blood cell growth factors may help the patient tolerate the side effects of higher doses of chemotherapy. People with CLL should discuss the risks and benefits of treatment with blood cell growth factors with their doctors.
Growth factors used to improve low white cell counts. CLL-related low blood counts are often corrected by CLL therapy. Sometimes the use of white cell growth factors can help people with CLL who have a long period of low white cell counts after treatment. Examples of white cell growth factors are Neupogen® or Neulasta® (also called "G-CSF") and Leukine® (also called "GM-CSF").
Other Treatments Radiation therapy is not a common treatment for CLL. This treatment uses x-rays or other high-energy rays to kill cancer cells. It is sometimes used to treat a person with CLL who has an enlarged (swollen) lymph node, spleen or other organ that is blocking the function of a neighboring body part, such as the kidney or the throat. Splenectomy, surgery to remove the spleen, is used only when a patient's spleen is affected by CLL. CLL cells can enlarge the spleen and cause discomfort in some patients with CLL. Also, an enlarged spleen may lower the patient's blood cell counts to dangerous levels. Splenectomy is helpful for some people with CLL if the spleen is very enlarged as a result of the disease. The operation may improve blood cell counts. Side Effects of Treatment CLL patients should talk with the doctor about side effects before they begin treatment. Patients react to CLL treatment in different ways. Sometimes patients have no side effects. Sometimes side effects are mild and last only a short time. Other side effects may be serious and last a long time. Most side effects go away when patients complete treatment. Some possible side effects of CLL treatment include
Treatment for Relapsed or Refractory CLL
"Relapsed CLL" is the term for CLL that responded to therapy but then stopped responding six or more months after therapy. "Refractory CLL" is the term used to describe CLL that either does not result in a remission (but may be stable) or CLL that gets worse within six months of the last treatment. People who are treated for relapsed or refractory CLL often have good quality years of remission after more treatment. Treatment options for people with relapsed or refractory CLL are generally the same as treatment for newly diagnosed people. But, some patients who have refractory CLL may have These patients may have CLL that does not respond to standard drug therapy. They should speak to their doctors about treatment in a clinical trial. back to top Clinical trials are used to study new drugs, new treatments or new uses for approved drugs or treatments. There are a growing number of CLL clinical trials for adults of all ages including newly diagnosed patients, patients with relapsed or refractory CLL and patients with CLL who continue treatment after remission (maintenance).
Some specific new drug therapies under study in clinical trials for people with CLL include Ofatumumab (Arzerra®) - Ofatumumab is an investigational, monoclonal antibody that targets CD20, a key target in CLL therapy because it is expressed on most B cells in people with CLL. Lumiliximab - This is an antibody that targets CD23 on the surface of CLL cells. It is being studied in clinical trials in combination with fludarabine, cyclophosphamide and rituximab (FCR) to see if it enhances the activity of FCR for the treatment of patients with relapsed CLL. Flavopiridol - Flavopiridol is being studied in people with high-risk genetic features whose CLL has responded to few, if any standard treatments. Lenalidomide (Revlimid) - This is an immunomodulatory drug that stimulates a person's own immune system to attack cancer cells. It is being evaluated in CLL trials to determine if lenalidomide given as a maintenance therapy is safe and effective in further improving the quality and duration of the response to treatment. Stem cell transplants are also under study in clinical trials. An allogeneic stem cell transplant is a type of stem cell transplant used to treat some diseases. This is a treatment to restore a person's marrow. Allogeneic stem cell transplantation is under study for people with high-risk CLL. It may be a good treatment for some people younger than 55 years who are not doing well with other treatments. The person also needs to have a "matched" stem cell donor. With an allogeneic stem cell transplant, stem cells from a donor are used. The donor can be a brother or sister. Or the donor can be another person with stem cells that "match" the stem cells of the transplant patient. First, the transplant patient is given high-dose chemotherapy and/or radiation therapy. Then stem cells from the donor are infused into to the transplant patient's blood. An allogeneic stem cell transplantation has a high risk of serious complications. Your doctor will explain the benefits and the risks if transplantation is suggested for you. Doctors are working to make allogeneic stem cell transplants safer. A type of transplant called a "reduced-intensity transplant" is under study. A reduced-intensity transplant uses lower doses of chemotherapy than a standard allogeneic stem cell transplant. This treatment is also called a nonmyeloablative transplant. Older and sicker patients may be helped by this treatment. Ask your doctor if treatment in a clinical trial is right for you. You can also call the Information Resource Center for information about clinical trials or use TrialCheck®, the free LLS-supported clinical trials service. back to top People with CLL have a range of responses after treatment. Talk to your doctor about the results of your treatment. Your doctor may use the following terms to talk about your response to treatment: Remission - No sign of disease; sometimes the terms "complete remission" (or complete response) or "partial remission" (or partial response) are used. Complete response (CR) - No sign of disease for at least two months after the end of treatment; normal blood counts and hemoglobin greater than 11 g/dL without transfusions; no CLL signs or symptoms. Partial response (PR) - At least a 50 percent reduction in the number of blood lymphocytes and in lymph node and spleen enlargement; one or more of the following must also be maintained for at least two months: platelets greater than 100,000/ul; hemoglobin greater than 11 g/dL; or a 50 percent improvement over pretreatment red cell or platelet counts without transfusions. Stable disease - This means the person's CLL is not growing, but the person does not have a complete or partial response. Progressive disease - The signs of progressive disease are at least one of the following:
- An increase of at least 50% in lymphocyte count or other signs of high-risk CLL.
- An increase of at least 50% in liver or spleen size or a newly enlarged liver or spleen.
- An increase of at least 50% in the combined size of at least two lymph nodes, in two exams in a row, done two weeks apart.
- New appearance of enlarged lymph nodes.
Follow-Up Care After CLL treatment, patients need to see their doctors for physical exams and blood tests. Bone marrow tests or FISH may be needed from time to time. The doctor may recommend longer periods of time between follow-up visits if a person continues to be free of CLL signs or symptoms. Minimal Residual Disease (MRD) Some people with CLL have a very low level of remaining CLL cells after treatment. The remaining CLL ells are called "minimal residual disease" (MRD). MRD cannot be detected by the usual blood and marrow tests. The tests to detect MRD in people with CLL are "four-color cell flow cytometry" and polymerase chain reaction (PCR). These tests may help the doctor to identify the need for more treatment. back to top
It is important to choose a doctor who specializes in treating CLL and who knows about the most up-to-date treatments. This type of specialist is usually called a "hematology oncologist." Or your local cancer specialist can work with a CLL specialist. You can talk with your doctor about how he or she plans to treat your CLL. This will help you to be actively involved in your care and to make decisions. It may be helpful to write down questions to ask your doctor. You can also write down or record your doctor's answers and review them later. You may want to bring a family member or friend with you to the doctor. This person can listen, take notes and offer support. Click here for more information on discussing treatment options and other concerns with your specialist. back to top The Leukemia & Lymphoma Society (LLS) offers ongoing education programs featuring leading leukemia specialists discussing the latest advances in the diagnosis and treatment of leukemia. Visit the Leukemia Education Series page to find out about our free upcoming and archived programs. You can also view, print or order the LLS free publications The CLL Guide: Information for Patients and Caregivers and Chronic Lymphocytic Leukemia. Visit the Leukemia Facts & Statistics page for more information on leukemia incidence and survival rates. back to top The Leukemia & Lymphoma Society
1311 Mamaroneck Ave.
White Plains, NY 10605 or call the Information Resource Center at (800) 955-4572. | 
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