Leukemia - B-CellLast Updated: July 12, 2011 This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/11 Overview
About leukemia Leukemia is a cancer of the blood cells. Leukemia begins when normal blood cells change and grow uncontrollably. Blood cells are made in the bone marrow, the spongy tissue inside the larger bones in the body. There are different types of blood cells, including red blood cells that carry oxygen throughout the body, white cells that fight infection, and platelets that help the blood to clot. Types of leukemia are named after the specific blood cell that becomes cancerous, such as the lymphoid cells (white blood cells of the immune system) or the myeloid cells (cells of the bone marrow which develop into cells that fight bacterial infections). There are four main types of leukemia in adults:
About PLL and HCL There are other, less common types of leukemia, but they are generally subcategories of one of the four main categories. This section focuses on prolymphocytic leukemia (PLL) and hairy cell leukemia (HCL), both of which are chronic B-cell leukemias. B cells are a specific type of lymphocyte that normally make antibodies for the immune system. In PLL, many immature lymphocytes, or prolymphocytes, are found in the blood. This type of leukemia may occur by itself, together with CLL, or CLL may turn into into PLL. HCL is a slow-growing form of leukemia. It is called “hairy cell” because the abnormal lymphocytes have projections that look like hair when seen under a microscope. As these cells multiply, they accumulate in the bone marrow, blood, and spleen. Because these lymphocytes are abnormal, they do not work normally to fight disease and infection, and eventually may crowd out the normal cells. HCL often responds well to treatment. Find out more about basic cancer terms used in this section. Looking for More of an Overview? If you would like additional introductory information, explore these related items on Cancer.Net:
Or, choose “Next” (below, right) to continue reading this detailed section. To select a specific topic within this section, use the icon panel located on the right side of your screen. Statistics
This year, an estimated 44,600 people of all ages (25,320 men and 19,280 women) in the United States will be diagnosed with leukemia. HCL accounts for about 2% of all leukemia. An estimated 14,570 people (8,520 men and 6,050 women) in the United States will be diagnosed with CLL. There are no current estimates for PLL. Cancer statistics should be interpreted with caution. These estimates are based on data from thousands of people with this type of cancer in the United States, but the actual risk for a particular individual may differ. It is not possible to tell a person how long he or she will live with leukemia. Statistics adapted from the American Cancer Society's publication, Cancer Facts and Figures 2011. Risk Factors
A risk factor is anything that increases a person’s chance of developing cancer. Although risk factors can influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop cancer, while others with no known risk factors do. However, knowing your risk factors and talking about them with your doctor may help you make more informed lifestyle and health care choices. In general, leukemia can be caused by a genetic mutation (change) that is inherited (passed from generation to generation within a family) or environmental factors, such as smoking, or chemical or radiation exposure. However, most often the cause of leukemia is not known. The following factors may raise a person’s risk of developing PLL or HCL: Age. HCL occurs most often in people between age 40 and 70. Gender. Men are five times more likely to develop HCL than women. Race/Ethnicity. HCL is more common in white people and Ashkenazi Jewish men. PLL and HCL are rare in people of Japanese or Southeast Asian descent. Symptoms and Signs
People with B-cell leukemia may experience the following symptoms or signs. Sometimes, people with B-cell leukemia do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not cancer. If you are concerned about a symptom or sign on this list, please talk with your doctor.
Your doctor will ask you questions about the symptoms you are experiencing to help find out the cause of the problem, called a diagnosis. This may include how long you’ve been experiencing the symptoms(s) and how often. If leukemia is diagnosed, relieving symptoms and side effects remains an important part of care and treatment. This may also be called symptom management, palliative care, or supportive care. Be sure to talk with your health care team about symptoms you experience, including any new symptoms or a change in symptoms. Diagnosis
Doctors use many blood and bone marrow tests to diagnose leukemia and to find out the extent to which it may have spread. Although a patient’s signs and symptoms may cause a doctor to suspect leukemia, it is diagnosed only by blood tests and bone marrow evaluations. Some tests may also determine which treatments may be the most effective. Your doctor may consider these factors when choosing a diagnostic test:
The following tests may be used to diagnose PLL and HCL: Blood tests. The diagnosis of PLL or HCL begins with a blood test, called a complete blood count (CBC), that measures the numbers of different types of cells in a person's blood. If the blood contains many white blood cells, B-cell leukemia may be suspected. Blood tests. These two procedures are similar and often done at the same time. Bone marrow has both a solid and a liquid part. A bone marrow biopsy is the removal of a small amount of solid tissue using a needle. An aspiration removes a sample of fluid with a needle. The sample(s) are then analyzed by a pathologist (a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease) to determine the number and type of abnormal cells. A common site for the bone marrow biopsy and aspiration is the pelvic bone, which is located in the lower back by the hip. The skin is usually numbed with medication beforehand, and other types of anesthesia may be used. Immunophenotyping. Immunophenotyping is the examination of antigens, a specific type of protein, on the surface of the leukemia cells. Immunophenotyping allows the doctor to confirm the exact type of leukemia. Cytogenetics. Cytogenetics is the examination of the leukemia cells for chromosome (long strands of genes) abnormalities. It helps doctors confirm the diagnosis and may help to determine the person’s prognosis (chance of recovery). Computed tomography (CT or CAT) scan. A CT scan creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. Sometimes, a contrast medium (a special dye) is injected into a patient’s vein to provide better detail. A CT scan also shows enlarged lymph nodes or a swollen spleen. Find more about what to expect when having common tests, procedures, and scans. After these diagnostic tests are done, your doctor will review all of the results with you. If the diagnosis is leukemia, these results also help the doctor describe the cancer. Learn more about the first steps to take after a diagnosis of cancer. Staging
Staging is a way of describing a cancer, such as where it is located, if or where it has spread, and whether it is affecting the functions of other organs in the body. Doctors use diagnostic tests to determine the cancer's stage, so staging may not be complete until all of the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient's prognosis. There are different stage descriptions for different types of cancer. Unlike most solid tumors, HCL and PLL do not have a formal staging system. Talk with your doctor for details on how your specific disease will be evaluated. Treatment
This section outlines treatments that are the standard of care (the best proven treatments available) for this specific type of cancer. When making treatment plan decisions, patients are also encouraged to consider clinical trials as an option. A clinical trial is a research study to test a new treatment to evaluate whether it is safe, effective, and possibly better than standard treatment. Your doctor can help you review all treatment options. For more information, see the Clinical Trials and Current Research sections. Treatment overview In cancer care, different types of doctors often work together to create a patient’s overall treatment plan that combines different types of treatments. This is called a multidisciplinary team. The goal of treatment for leukemia is remission (absence of cancer symptoms; also called “no evidence of disease” or NED). Descriptions of the most common treatment options for B-cell leukemia are listed below. Treatment options and recommendations depend on several factors, including the type of leukemia, possible side effects, and the patient’s preferences and overall health. Learn more about making treatment decisions. Chemotherapy Chemotherapy is the use of drugs to kill cancer cells, usually by stopping the cancer cells’ ability to grow and divide. Systemic chemotherapy is delivered through the bloodstream to reach cancer cells throughout the body. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating cancer with medication, or a hematologist, a doctor who specializes in treating blood disorders. A chemotherapy regimen (schedule) usually consists of a specific number of cycles given over a set period of time. A patient may receive one drug at a time or combinations of different drugs at the same time. Drugs that are used to treat HCL include cladribine (Leustatin) and pentostatin (Nipent). For most people with HCL, both cladribine and pentostatin can cause remissions that last for many years and are the standard initial treatment for the disease. PLL is treated with combination chemotherapy, meaning more than one drug is used. Several regimens may be used. The regimens are chosen by the type of chemotherapy the patient may have received previously and the possible side effects. The side effects of chemotherapy depend on the individual and the dose used, but they can include fatigue, risk of infection, nausea and vomiting, loss of appetite, and diarrhea. These side effects usually go away once treatment is finished. Learn more about chemotherapy and preparing for treatment. The medications used to treat cancer are continually being evaluated. Talking with your doctor is often the best way to learn about the medications prescribed for you, their purpose, and their potential side effects or interactions with other medications. Learn more about your prescriptions by using searchable drug databases. Immunotherapy Immunotherapy (also called biologic therapy) is designed to boost the body's natural defenses to fight the cancer. It uses materials made either by the body or in a laboratory to bolster, target, or restore immune system function. Immunotherapy for HCL includes recombinant interferon alpha (Alferon, Intron A, Roferon-A). Learn more about immunotherapy. Targeted therapy Targeted therapy is a treatment that targets the cancer’s specific genes, proteins, or the tissue environment that contributes to cancer growth and survival. This type of treatment blocks the growth and spread of cancer cells while limiting damage to normal cells, usually leading to fewer side effects than other cancer medications. Recent studies show that not all cancers have the same targets. To find the most effective treatment, your doctor may run tests to identify the genes, proteins, and other factors involved in your leukemia. As a result, doctors can better match each patient with the most effective treatment whenever possible. In addition, many research studies are taking place now to find out more about specific molecular targets and new treatments directed at them. Learn more about targeted treatments. Monoclonal antibodies are a type of targeted therapy that target specific proteins on the surface of the cancer cell. Rituximab (Rituxan) is an antibody directed against the surface protein CD20. BL22 immunotoxin is an antibody attached to a toxin that is designed to attach to the surface protein CD22 and deliver the toxin to the cancer cell. Both of these have been used to treat HCL that did not go into remission after initial therapy. However, BL22 is not approved by the U.S. Food and Drug Administration (FDA) for the treatment of PLL and is only available in clinical trials. Alemtuzumab (Campath) is an antibody that targets the surface protein CD52 and has been used to treat PLL. Surgery Surgery to remove the spleen (splenectomy), which also makes white blood cells, may be done for some patients. A surgical oncologist is a doctor who specializes in treating cancer using surgery. Learn more about cancer surgery. Stem cell transplantation/bone marrow transplantation A stem cell transplant is a medical procedure in which diseased bone marrow is replaced by highly specialized cells, called hematopoietic stem cells. Hematopoietic stem cells are found both in the bloodstream and in the bone marrow. Today, this procedure is more commonly called a stem cell transplant, rather than bone marrow transplant, because it is the blood stem cells that are typically being transplanted, not the actual bone marrow tissue. Before recommending transplantation, doctors will talk with the patient about the risks of this treatment and consider several other factors, such as the type of cancer, results of any previous treatment, and patient’s age and general health. Transplantation is a higher-risk procedure. It is not used often for people with B-cell leukemia because it is not always an effective therapy for these diseases and because many patients with B-cell leukemia are older and the risks of the procedure are higher. There are two types of stem cell transplantation depending on the source of the replacement blood stem cells: allogeneic (ALLO) and autologous (AUTO). ALLO transplantation is sometimes considered for younger patients with PLL when chemotherapy was not effective. However, AUTO transplantation is rarely considered for patients with PLL or HCL. In an ALLO transplant, stem cells are obtained from a donor whose tissue matches the patient’s on a genetic level; this testing is called HLA-typing. Most often, a patient’s brother or sister serves as the donor, although unrelated donors can serve as the donor too. Millions of people worldwide have volunteered to donate stem cells for patients who do not have matched family members; your health care team will search a computer registry to look for a match. In addition, a donation of stem cells derived from umbilical cord blood is sometimes considered if family donors are not available. In an AUTO transplant, the patient’s own stem cells are used. The stem cells are obtained from the patient when he or she is in remission from previous treatment. The stem cells are then frozen until they are needed, usually after the high-dose treatment (explained below) is completed. In both types, the goal of transplantation is to destroy disease cells in the marrow, blood, and other parts of the body and allow replacement blood stem cells create healthy bone marrow. In most stem cell transplants, the patient is treated with high doses of chemotherapy and/or radiation therapy to destroy as many cancer cells as possible. This also destroys the patient’s bone marrow tissue and suppresses the patient’s immune system so that, in an ALLO transplant, the donor cells are not rejected by the body. After the high-dose treatment is given, blood stem cells are infused into the patient’s vein to replace the bone marrow and restore normal blood counts from donor cells. Sometimes, ALLO transplants can also be performed after giving lower doses of chemotherapy and/or radiation therapy that are still sufficient to suppress the immune system and allow growth of the donor cells. (These transplants, sometimes termed “mini-transplants” or reduced intensity transplants, have less immediate side effects, allowing the procedure to be used for older patients.) For both ALLO and AUTO transplants, the replacement cells engraft (begin to make new blood cells) and turn into healthy, blood-producing tissue in 10 days to three weeks. Destroying the patient’s own marrow reduces the body’s natural defenses, temporarily leaving the patient at an increased risk of infection. Until the patient’s immune system is back to normal, patients may need antibiotics and blood transfusions, and they need to follow certain safety restrictions provided by the health care team to help avoid infections. In an ALLO transplant, another major risk is that the donor’s cells will recognize the patient’s body as foreign, causing graft-versus-host disease (GVHD). GVHD may be a serious complication of allogeneic transplants and can be fatal. Other side effects may include liver problems, diarrhea, infections, and rashes. However, GVHD can also be a benefit because the donor cells can recognize the disease cells as foreign and destroy these cells, a mechanism that is one of the major reasons why ALLO transplantation generally works so well over the long term. The risk of GVHD can be reduced with exact HLA-type matching and the use of preventive drugs. In an AUTO transplant, there is little risk of GVHD because the replacement stem cells are the patient’s own cells. However, there is a risk in an autologous transplant that some of the cells that are put back into the patient could still contain disease. Learn more about bone marrow and stem cell transplantation. Recurrent B-cell leukemia Once your treatment is complete and there is a remission, talk with your doctor about the possibility of the cancer returning. Many survivors feel worried or anxious that the cancer will come back. Learn more about coping with this fear. If the cancer does return after the original treatment, it is called recurrent leukemia. When this occurs, a cycle of testing will begin again to learn as much as possible about the recurrence. After testing is done, you and your doctor will talk about your treatment options. Often the treatment plan will include the therapies described above (such as chemotherapy, immunotherapy, targeted therapy, and stem cell transplantation) but may be used in a different combination or given at a different pace. For instance, cladribine and pentostatin can be successful again to treat HCL if it recurs after a long period of remission. Your doctor may also suggest clinical trials that are studying new ways to treat this type of recurrent cancer. People with recurrent cancer often experience emotions such as disbelief or fear. Patients are encouraged to talk with their health care team about these feelings and ask about support services to help them cope. Learn more about dealing with cancer recurrence. Refractory leukemia If the leukemia continues to worsen despite treatment, it is called refractory or resistant leukemia. Patients with this diagnosis are encouraged to talk with doctors who are experienced in treating this type of leukemia, because there can be different opinions about the best treatment plan. Learn more about seeking a second opinion before starting treatment, so you are comfortable with the treatment plan chosen. This discussion may include clinical trials studying new treatments. For HCL, your health care team may recommend a treatment plan that includes rituximab and/or BL22 immunotoxin. In addition to treatment to slow, stop, or eliminate the cancer (also called disease-directed treatment), an important part of cancer care is relieving a person’s symptoms and side effects. It includes supporting the patient with his or her physical, emotional, and social needs, an approach called palliative or supportive care. People often receive disease-directed therapy and treatment to ease symptoms at the same time. If disease-directed treatment is not successful, then further therapy may not be recommended. This situation is very stressful, and it may be difficult to discuss. However, it is important to have open and honest conversations with your doctor and health care team to express your feelings, preferences, and concerns. The health care team is there to help, and many team members have special skills, experience, and knowledge to support patients and their families. Learn more about advanced cancer care planning. Find out more about common terms used during cancer treatment. About Clinical Trials
Doctors and scientists are always looking for better ways to treat patients with B-cell leukemia. To make scientific advances, doctors create research studies involving people, called clinical trials. Many clinical trials are focused on new treatments, evaluating whether a new treatment is safe, effective, and possibly better than the current (standard) treatment. These types of studies evaluate new drugs, different combinations of existing treatments, new approaches to radiation therapy or surgery, and new methods of treatment. Patients who participate in clinical trials are often among the first to receive new treatments before they are widely available. However, there is no guarantee that the new treatment will be safe, effective, or better than a standard treatment. There are also clinical trials that study new ways to ease symptoms and side effects during treatment and manage the late effects that may occur after treatment. Talk with your doctor about clinical trials regarding side effects. In addition, there are ongoing studies about ways to prevent the disease. Patients decide to participate in clinical trials for many reasons. For some patients, a clinical trial is the best treatment option available. Because standard treatments are not perfect, patients are often willing to face the added uncertainty of a clinical trial in the hope of a better result. Other patients volunteer for clinical trials because they know that these studies are the only way to make progress in treating B-cell leukemia. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with B-cell leukemia. Sometimes people have concerns that, by participating in a clinical trial, they may receive no treatment by being given a placebo or a “sugar pill.” The use of placebos in cancer clinical trials is rare. When a placebo is used in a study, it is done with the full knowledge of the participants. Find out more about placebos in cancer clinical trials. To join a clinical trial, patients must participate in a process known as informed consent. During informed consent, the doctor should list all of the patient’s options so that the person understands how the new treatment differs from the standard treatment. The doctor must also list all of the risks of the new treatment, which may or may not be different from the risks of standard treatment. Finally, the doctor must explain what will be required of each patient in order to participate in the clinical trial, including the number of doctor visits, tests, and the schedule of treatment. Learn more about clinical trials, including patient safety, phases of a clinical trial, deciding to participate in a clinical trial, questions to ask the research team, and links to find clinical trials. For specific topics being studied for B-cell leukemia, learn more in the Current Research section. Patients who participate in a clinical trial may stop participating at any time for any personal or medical reason. This may include that the new treatment is not working or there are serious side effects. It is important that patients participating in a clinical trial talk with their doctor and researchers about who will be providing their treatment and care during the clinical trial, after the clinical trial ends, and/or if the patient chooses to leave the clinical trials before it ends. Side Effects
Cancer and its treatment can cause a variety of side effects. However, doctors have made major strides in recent years in reducing pain, nausea and vomiting, and other physical side effects of cancer treatments. Many treatments used today are less intensive but as effective as treatments used in the past. Doctors also have many ways to provide relief to patients when such side effects occur. Fear of treatment side effects is common after a diagnosis of cancer, but it may be helpful to know that preventing and controlling side effects is a major focus of your health care team. Before treatment begins, talk with your doctor about possible side effects of the specific treatments you will be receiving. The specific side effects that can occur depend on a variety of factors, including the type of cancer, its location, the individual treatment plan (including the length and dosage of treatment), and your overall health. Common side effects for each treatment option are described in detail within the Treatment section. Ask your doctor which side effects are most likely to happen (and which are not), when side effects are likely to occur, and how they will be addressed by the health care team. Also, be sure to communicate with the doctor about side effects you experience during and after treatment. Care of a patient’s symptoms and side effects is an important part of a person’s overall treatment plan; this is called palliative or supportive care. It helps people with cancer at any stage of illness be as comfortable as possible. Learn more about the most common side effects of cancer and different treatments, along with ways to prevent or control them. Be sure to talk with your doctor about the level of caregiving you may need during treatment and recovery, as family members and friends often play an important role in the care of a person with B-cell leukemia. Learn more about caregiving. In addition to physical side effects, there may be psychosocial (emotional and social) effects as well. For many patients, a diagnosis of B-cell leukemia is stressful and can bring difficult emotions. Patients and their families are encouraged to share their feelings with a member of their health care team who can help with coping strategies. Learn more about the importance of addressing such needs, including concerns about managing the cost of your cancer care. A side effect that occurs more than five years after treatment is called a late effect. Treatment of late effects is an important part of survivorship care. Learn more about late effects or long-term side effects by reading the After Treatment section or talking with your doctor. After Treatment
After treatment for B-cell leukemia ends, talk with your doctor about developing a follow-up care plan. This plan may include regular physical examinations, blood tests, bone marrow biopsies, and possibly scans or other imaging tests to monitor your recovery for the coming months and years. People experiencing a long-term remission are encouraged to follow cancer screening recommendations for the general population. Several long-term side effects from leukemia treatment may occur:
ASCO offers cancer treatment summary forms to help keep track of the cancer treatment you received and develop a survivorship care plan once treatment is completed. People recovering from B-cell leukemia are encouraged to follow established guidelines for good health, such as, maintaining a healthy weight, not smoking, eating a balanced diet, and having recommended cancer screening tests. Talk with your doctor to develop a plan that is best for your needs. Moderate physical activity can help rebuild your strength and energy level. Your doctor can help you create an appropriate exercise plan based upon your needs, physical abilities, and fitness level. Learn more about the next steps to take in survivorship, including making positive lifestyle changes. Find out more about common terms used after cancer treatment is complete. Current Research
Doctors are working to learn more about B-cell leukemia, ways to prevent it, how to best treat it, and how to provide the best care to people diagnosed with this disease. The following areas of research may include new options for patients through clinical trials. Always talk with your doctor about the diagnostic and treatment options best for you. Targeted therapy. New treatments are being tested in clinical trials, including those that use angiogenesis inhibitors and monoclonal antibodies. Angiogenesis inhibitors are focused on stopping angiogenesis, which is the process of making new blood vessels. As discussed in the Treatment section, BL22 and LMB-2 are monoclonal antibodies linked to toxins that are designed to attach to the surface proteins of leukemia cells. Doctors are using these and other monoclonal antibodies in clinical trials for people with HCL when other treatments no longer work. Supportive care. Clinical trials are underway to find better ways of reducing symptoms and side effects of current B-cell leukemia treatments in order to improve patients’ comfort and quality of life. Learn more about common statistical terms used in cancer research. Looking for More about Current Research? If you would like additional information about the latest areas of research regarding leukemia, explore these related items:
Or, choose “Next” (below, right) to continue reading this detailed section. Questions to Ask the Doctor
Talking often with the doctor is important to make informed decisions about your health care. These suggested questions are a starting point to help you learn more about your cancer care and treatment. You are also encouraged to ask additional questions that are important to you.
Patient Information Resources
In addition to Cancer.Net, there are other sources of information about this type of cancer available online. Cancer.Net maintains a list of national, not-for-profit organizations that may be helpful in finding additional information, services, and support. As always, be sure to talk with your doctor about questions you may have about information you find about this disease. View organizations that offer information on this specific type of cancer. |
