Chronic lymphocytic leukemia/small lymphocytic lymphoma is unlike other cancers in the following ways:
• Presently, there is no cure for CLL/SLL. The overall goal of leukemia treatment is to bring about a remission. During remission, the signs and symptoms of the cancer disappear; however, the cancer may still be in the body. The reappearance of the signs and symptoms during a remission is called a relapse. All patients who achieve a remission are expected to eventually relapse. However, further treatment can bring about subsequent remissions.
• Treatment for CLL/SLL is not always needed. In some people, symptoms may not develop for several years, if at all. In others, CLL causes significant symptoms or can even be lethal. Choosing a treatment option involves balancing the risks of the disease with those of potential side effects of treatment. Deciding when to begin treatment is fundamental for surviving this disease.
• With CLL/SLL, there is no tumor that can be removed. The cancer starts in the white blood cells that circulate throughout the body, including the bone marrow and lymph nodes. The bone marrow cannot be removed because it occupies the center of all the major bones and produces the blood cells that are needed by the rest of the body. Therefore, the treatment of leukemia focuses on destroying the abnormal leukemia cells in the body, which typically is associated with a host of potential side effects.
The most important decision facing the patient diagnosed with CLL is whether active treatment is necessary. The treatment for CLL is guided by the patient's age and overall health, the stage of the disease, and the presence of biological markers that are indicative of how rapidly CLL/SLL will progress.
Most experts believe that the presence or absence of biological risk factors is as important as the staging system for determining treatment, particularly for people in the earliest stages of CLL. These include the location and type of abnormalities in the chromosomes as determined by fluorescent in situ hybridization, the IgVH gene mutation status, and the concentrations of cellular proteins such as ZAP-70 and CD38. However, scientists and doctors are just beginning to verify the role of chromosomal mutations in directing treatment for patients with CLL/SLL.
Typically, the best outcome occurs when you and your doctor work together to develop a treatment strategy. A clear understanding of your condition and clear communication with your doctor can help ensure that you are receiving the best possible treatment and supportive care. Diet, sleep, and medications to control pain and side effects are important components in the successful treatment of CLL/SLL.
Questions you may want to ask your doctor when selecting a treatment include:
- What stage is my CLL?
- Which biological markers do I have, and what is their significance?
- What are the treatment options?
- Which treatment do you recommend, and why?
- How will the treatment affect my survival?
- When will treatment begin, how long will it last, and what is the schedule?
- Does this treatment require hospitalization?
- Will I be able to go to work or school during treatment?
- How will the treatment make me feel?
- What are the side effects and risks of the treatment?
- How long could the side effects last? Could they be permanent?
- Will I need medications to control pain or other side effects?
- How do we know if it's working?
- Is there anything I can do to prepare?
This section includes information on:
- Watch and wait
- Biological therapy: monoclonal antibodies
- Radiation therapy
- Blood and bone marrow transplant
- Experimental therapy in a clinical trial
- Supportive care
For people with early-stage chronic lymphocytic leukemia/small lymphocytic lymphoma with no adverse biological markers or troublesome symptoms, the risks associated with the disease may not warrant the risks and discomfort of treatment. Instead, their doctor may recommend careful observation ("watchful waiting" or "expectant management"). Early treatment of patients with CLL using chemotherapy has never been shown to improve survival and can increase the risk of complications.
If you and your doctor choose to watch and wait, you will need regular checkups to monitor your health. No active treatment will be undertaken. Checkups are necessary every three to six months to ensure that treatment is started promptly, if it is needed. Regular checkups typically include a detailed history and physical examination and a blood analysis. However, if symptoms develop, do not wait for your regular checkup; report the symptoms to your healthcare team immediately.
A combination of chemotherapy with monoclonal antibodies (chemoimmunotherapy) is the most widely used initial treatment for chronic lymphocytic leukemia/small lymphocytic lymphoma. Chemotherapy is also used as a conditioning therapy prior to a blood and bone marrow transplant.
Hematologists usually recommend a combination of drugs administered over time for people with CLL/SLL. This is often more effective than a single drug, as drugs attack the cancer cells in several different ways. Doctors must also consider side effects of each drug and any potential interactions among the drugs.
During chemotherapy, drugs that damage the DNA in the body's cells are introduced into the bloodstream. For patients with CLL/SLL, the chemotherapy drugs are usually swallowed in pill form or injected by needle into a vein.
Chemotherapy drugs do not distinguish between healthy cells and cancer cells. Most of the side effects of chemotherapy are caused by the damage to normal cells. Each time chemotherapy is given, the doctor is balancing destroying the cancer cells with sparing the normal cells. The side effects are usually temporary, and medications often can be given to lessen them. The cells most vulnerable to chemotherapy are those that reproduce rapidly, including the bone marrow and the lining of the reproductive and digestive tracts.
Common side effects of chemotherapy given for treatment of CLL/SLL include:
- Lowered resistance to infection because of low white blood cell counts
- Easy bruising or bleeding caused by low blood platelets
- Fatigue resulting from low red blood cells
- Loss of appetite, nausea, and vomiting
To minimize your risk of infection, it may be helpful to reduce your exposure to germs by:
- Washing your hands often
- Avoiding uncooked fruits and vegetables and other foods that might carry germs
- Avoiding fresh flowers and plants because they carry mold
- Asking people to wash their hands when they come in contact with you
- Avoiding large crowds and people who are sick (wearing a surgical mask offers some protection in these situations)
Other medications, including vitamins and nonprescription medicines, can interact with chemotherapy and potentially make the drugs less effective or exacerbate their side effects. Therefore, it is important to tell your doctor about all medicines that you are taking, including herbal or dietary supplements.
Common chemotherapy drugs. Chlorambucil (Leukeran) and cyclophosphamide (Cytoxan) are alkylating agents that damage cells' DNA and eventually kill the cells. Alkylating agents are also used to treat kidney disease and many other cancers, including cancer of the ovaries and breast, as well as many other medical conditions. Fludarabine (Fludara) and pentostatin (Nipent) are chemotherapy drugs that are particularly toxic to both malignant and normal lymphocytes.
Patients with chronic lymphocytic leukemia/small lymphocytic lymphoma are often treated with monoclonal antibodies, special proteins that are developed in a laboratory. They are similar to the antibodies that are produced by the immune system but are created to specifically react with certain types of cancer cells. These antibodies help bolster your immune system to destroy the cancer. Biological therapy is also used to treat acute lymphocytic leukemia.
Alemtuzumab (Campath) is a monoclonal antibody that is very effective in the treatment of patients with CLL even when their disease is no longer responding to standard chemotherapy treatments. It is usually given daily or several times a week by either subcutaneous or intravenous injection. The main side effects are fever and chills during the first few injections and increased susceptibility to infections because of destruction of the body's normal lymphocytes that fight infection.
Rituximab (Rituxan) is another monoclonal antibody often used in combination with chemotherapy. This drug commonly causes a "first dose" reaction including fever, chills, a drop in blood pressure, and shortness of breath, but this can be alleviated by careful administration and premedication. Rituximab also kills normal antibody-producing cells and can increase the risk of several infections.
Radiation therapy uses ionizing energy to damage the DNA of cells and bring about cell death. Radiation therapy is rarely used to treat patients with CLL/SLL. It is primarily part of the conditioning therapy to prepare the body for a blood and bone marrow transplant.
During treatment, the radiation is focused from a machine outside the body onto the whole body or onto an area where leukemia cells have accumulated. This "external beam" radiation therapy is like getting an X-ray but for a longer time. It usually involves daily treatments over several weeks. Newer types of external beam radiation are making radiation therapy safer and more effective.
Radiation therapy attacks cancer cells that are dividing, but it can also affect dividing cells of normal tissues. It is the damage to normal cells that causes side effects. Each time radiation therapy is given, the doctor is balancing destroying the cancer cells against sparing the normal cells. The main short-term side effects of radiation therapy are skin changes similar to sunburn, fatigue, and lowered resistance to infection. Many of these side effects can be lessened by supportive care. Discuss with your doctor how best to manage your side effects.
Patients should check with their doctors before taking any vitamins or nutrition supplements during radiation treatment. Certain vitamins, such as A, E, and C, act as antioxidants and prevent the formation of ions that damage DNA in cells. Some scientists believe that high doses of antioxidant vitamins may reduce the effectiveness of radiation therapy. No studies have thoroughly tested this theory.
A blood and bone marrow transplant (BMT) is used to resupply the bone marrow with healthy blood-forming stem cells and to provide healthy immune cells that can destroy the abnormal cells. BMT allows patients to receive the high doses of chemotherapy and radiation therapy needed to kill off leukemia cells—higher than they could normally tolerate.
Depending on the source of the stem cells, this procedure may be called a bone marrow transplant, a peripheral blood stem cell transplant, or a cord blood transplant. If the blood cells are derived from the blood circulating in the body, the procedure is referred to as a peripheral blood stem cell transplant. In a cord blood transplant, the stem cells come from a newborn baby's umbilical cord.
BMTs are classified based on the type of donor as well. The stem cells used are most often donated by a matched family member or an unrelated but matched donor; this is called an allogeneic transplant. A transplant from an identical twin is called a syngeneic transplant, and a transplant of your own stem cells is an autologous transplant. Autologous transplant is not an effective treatment for CLL/SLL.
For people with leukemia, the first step in a BMT is "conditioning," in which they undergo chemotherapy, sometimes combined with radiation therapy. Conditioning is designed to kill off the leukemia cells and suppress the immune system so that the body does not reject the transplanted bone marrow cells. After conditioning, donated stem cells are infused using an IV. No surgery is required for a BMT.
A BMT is a lengthy process that includes several phases. Complete recovery to normal blood cell counts and a fully functional immune system may take six months to one year. During this time, one's life and family relationships will be disrupted. Side effects of BMT can be significant and vary with the phase of treatment and the type of chemo or radiation therapy and type of donor cells. Some problems may appear even a year or more after healthy cells are infused.
For patients with CLL/SLL, the risk of serious complications from a standard BMT, including death, can be as high as 30 to 40 percent. Reduced-intensity BMT procedures that have fewer side effects can be an effective treatment option for some patients with CLL/SLL.
Clinical trials offer patients the opportunity to receive new therapies or new combinations of known therapies to treat their leukemia. A clinical trial is one of the final stages of a long and careful cancer research process. These studies are undertaken to find out whether promising approaches to diagnosing, preventing, and treating cancer are safe and effective. Several new drugs for treating chronic lymphocytic leukemia are currently being tested in clinical trials.
There are several ways to learn more about clinical trials. Your doctor can help you find a clinical trial that may be of benefit to you. The American Cancer Society also provides a clinical trials matching service for patients, family, and friends. To learn about this service, you can call the ACS cancer information center at (800) 227-2345 or visit the website. You can also get a list of current clinical trials by calling the National Cancer Institute's Cancer Information Service toll free at (800) 422-6237 or by visiting the NCI clinical trials website.
Supportive care is an important component of leukemia treatment. Supportive care can be as simple as proper diet and extra sleep or can involve medications to control the pain and side effects of treatment.
Last reviewed on 07/01/2008
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