Leukemia is a cancer of the blood cells. Types of leukemia are named after the specific blood cell that becomes cancerous, such as the lymphocytic cells (white blood cells of the immune system) or the myeloid cells (cells of the bone marrow, the spongy, red tissue in the inner part of large bones). There are four main types of leukemia in adults:
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), which are chronic B-cell leukemias. B cells are a specific type of lymphocyte that normally help produce antibodies for the immune system. In PLL, large numbers of immature lymphocytes, or prolymphocytes, are present in the blood. This type of leukemia may occur together with CLL, or CLL may transform 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 perform the normal work of lymphocytes in fighting disease and infection, and eventually may crowd out the normal cells. HCL responds well to treatment.
Statistics
In 2008, an estimated 44,270 people of all ages (25,180 men and 19,090 women) in the United States will be diagnosed with leukemia. HCL accounts for 2% of all leukemia that is diagnosed this year. An estimated 15,110 people (8,750 men and 6,360 women) in the United States will be diagnosed with CLL.
Cancer statistics should be interpreted with caution. These estimates are based on data from thousands of cases of this type of cancer in the United States each year, 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 2008.
A risk factor is anything that increases a person’s chance of developing cancer. Some risk factors can be controlled, such as smoking, and some cannot be controlled, such as age and family history. 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 communicating them to your doctor may help you make more informed lifestyle and health-care choices.
Leukemia can be caused by a hereditary genetic mutation or environmental factors (including smoking, chemical, or radiation exposure). However, many cases of leukemia have unknown causes.
The following factors can raise a person’s risk of developing PLL and HCL:
Age. HCL occurs primarily in people between 40 and 70.
Sex. Men are five times more likely to develop HCL than women.
Race. HCL is more common in white people and Ashkenazi Jewish men. PLL and HCL are rare in people of Japanese or Southeast Asian descent.
There have been no specific risk factors for the development of PLL or HCL identified at this time.
People with B-cell leukemia may experience the following symptoms. 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 on this list, please talk with your doctor.
Enlarged lymph nodes, in PLL (In HCL, hairy cells accumulate in the spleen and bone marrow and may not cause noticeable lymph node swelling.)
Tendency to bruise easily
Slow wound healing
Weight loss
Unexplained tiredness (fatigue)
Persistent, unexplained abdominal pain on the left side, due to an enlarged spleen
Doctors use many tests, such as blood tests and bone marrow tests, to diagnose leukemia and determine if it has metastasized (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:
Age and medical condition
The type of cancer
Severity of symptoms
Previous test results
The following tests may be used to diagnose PLL and HCL:
Blood tests. The diagnosis of PLL or HCL begins with a blood test to measure the counts of different types of cells in a person's blood. If the blood contains high levels of white blood cells, B-cell leukemia may be present.
Bone marrow biopsy. In a bone marrow biopsy, a doctor takes a sample of marrow, usually from the back of the patient’s hipbone, with a needle. The cells from the marrow, along with the cells from the blood, are analyzed by a pathologist (a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease).
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 chromosomal abnormalities. It assists in confirming the diagnosis and may help the doctor 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 puts 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 vein to provide better detail. A CT scan also shows enlarged lymph nodes or a swollen spleen.
Staging is a way of describing a cancer, such as where it is located, if or where it has spread, and if 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.
The treatment of HCL or PLL depends on the development of the disease, whether the cancer has spread, and the person’s overall health. In many cases, a team of doctors will work with the patient to determine the best treatment plan.
The goal of treatment for a blood cancer is to bring about a remission of the disease. A complete remission means the doctor can find no evidence of the disease.
Chemotherapy
Chemotherapy is the use of drugs to kill cancer cells. Systemic chemotherapy is delivered through the bloodstream, targeting cancer cells throughout the body. The side effects of chemotherapy depend on the individual and the dose used, but can include fatigue, risk of infection, nausea and vomiting, loss of appetite, and diarrhea. These side effects usually go away once treatment is finished.
Chemotherapy agents that are used to treat HCL include cladribine (Leustatin), pentostatin (Nipent), and interferon (Alferon N, Roferon-A, Intron A). In most people with HCL, both cladribine and pentostatin produce remissions that last for many years and are the standard initial therapy for the disease. If the initial response was long, these agents can be successful again in treating recurrent HCL (HCL that comes back after treatment).
PLL is treated with combination chemotherapy. Several regimens may be used. The regimens are selected according to the type of chemotherapy the patient may have received previously and any associated side effects.
The medications used to treat cancer are continually being evaluated. Talking with your doctor is often the best way to learn about the medications you've been prescribed, their purpose, and their potential side effects or interactions with other medications. Learn more about your prescriptions through Cancer.Net's Drug Information Resources, which provides links to searchable drug databases.
Monoclonal antibodies
Monoclonal antibodies are antibodies directed against specific proteins on the cell surface of the cancer cell. Rituximab (Rituxan) is an antibody directed against the surface protein CD20. BL22 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 in the treatment of HCL that did not go into remission after initial therapy (called refractory or resistant HCL). Alemtuzumab (Campath) is an antibody directed against the surface protein CD52 and has been used in the treatment of PLL.
Surgery
Surgery to remove the spleen (splenectomy), which also produces white blood cells, may be appropriate in some people.
Stem cell transplantation
Hematopoietic stem cells are special cells that can develop into different kinds of blood cells, such as red blood cells, white blood cells, or platelets. Stem cells are found both in the circulating blood and in the bone marrow, so this procedure may also be called a bone marrow transplantation. In a stem cell transplantation (SCT), the person is first treated with high doses of chemotherapy and/or radiation therapy to get rid of as many cancer (leukemia) cells as possible and to prevent the immune system from reacting to and rejecting the donated stem cells. After the high-dose therapy is given, stem cells obtained from a healthy donor (usually a sibling) are infused into the patient’s bloodstream. Within two to three weeks, these cells will mature into healthy, blood-producing tissue. Until that time, people may need antibiotics to prevent and treat infection and transfusions of red blood cells and platelets.
This type of SCT is called an allogeneic SCT, or an ALLO, because the new stem cells came from a donor. Allogeneic transplantation may be a considered a good treatment option in some patients, especially those who are younger. More recently, some transplants have been carried out using less-intensive therapies, referred to as reduced intensity conditioning or "mini" transplants, which have fewer short-term side effects. This allows transplants to be done in some older individuals.
An autologous transplantation (AUTO) is the use of the person’s own stem cells. The blood or bone marrow stem cells are removed from the person when he or she is in complete remission and then frozen. The person then receives the same treatment given for an allogeneic transplantation, and the frozen stem cells are thawed and injected back into the bloodstream to replace the destroyed marrow. Autologous SCT is rarely considered for patients with B-cell leukemia.
SCT is a higher-risk procedure and is not used frequently in people with B-cell leukemia because it is not a consistently effective therapy and because many patients with this disease are older and the risks of the procedure are higher.
Biologic therapy, or immunotherapy, attempts to fight the cancer by supplying a substance (either made by the body or produced in a laboratory) to boost the body’s natural defenses.
Cancer and cancer treatment can cause a variety of side effects; some are easily controlled and others require specialized care. Below are some of the side effects that are more common to B-cell leukemia and its treatments. For more detailed information on managing these and other side effects of cancer and cancer treatment, visit the Cancer.Net Managing Side Effects section.
Constipation. Constipation is the infrequent or difficult passage of stool. About 40% of patients in palliative care (care given to improve a patient’s quality of life) experience constipation, and about 90% of patients taking opioid medications (such as morphine) experience constipation. Constipation includes fewer bowel movements, stools that are abnormally hard, discomfort, or a feeling of incomplete rectal emptying. Patients with constipation can experience pain, swelling in the abdomen, loss of appetite, nausea and/or vomiting, inability to urinate, and confusion.
Fatigue (tiredness). Fatigue is extreme exhaustion or tiredness, and is the most common problem that people with cancer experience. More than half of patients experience fatigue during chemotherapy or radiation therapy, and up to 70% of patients with advanced cancer experience fatigue. Patients who feel fatigue often say that even a small effort, such as walking across a room, can seem like too much. Fatigue can seriously affect family activities and other daily activities, can make patients avoid or skip cancer treatments, and may even affect the will to live.
Hair loss (alopecia). A potential side effect of radiation therapy and chemotherapy is hair loss. Radiation therapy and chemotherapy cause hair loss by damaging the hair follicles responsible for hair growth. Hair loss may occur throughout the body, including the head, face, arms, legs, underarms, and pubic area. The hair may fall out entirely, gradually, or in sections. In some cases, the hair will simply thin-sometimes unnoticeably-and may become duller and dryer. Losing one's hair can be a psychologically and emotionally challenging experience and can affect a patient's self-image and quality of life. However, the hair loss is usually temporary, and the hair often grows back.
Infection. An infection occurs when harmful bacteria, viruses, or fungi (such as yeast) invade the body and the immune system is not able to destroy them quickly enough. Patients with cancer are more likely to develop infections because both cancer and cancer treatments (particularly chemotherapy and radiation therapy to the bones or extensive areas of the body) can weaken the immune system. Symptoms of infection include fever (temperature of 100.5°F or higher); chills or sweating; sore throat or sores in the mouth; abdominal pain; pain or burning when urinating or frequent urination; diarrhea or sores around the anus; cough or breathlessness; redness, swelling, or pain, particularly around a cut or wound; and unusual vaginal discharge or itching.
Mouth sores (mucositis). Mucositis is an inflammation of the inside of the mouth and throat, leading to painful ulcers and mouth sores. It occurs in up to 40% of patients receiving chemotherapy. Mucositis can be caused by chemotherapy directly, the reduced immunity brought on by chemotherapy, or radiation therapy to the head and neck area.
Nausea and vomiting. Vomiting, also called emesis or throwing up, is the act of expelling the contents of the stomach through the mouth. It is a natural way for the body to rid itself of harmful substances. Nausea is the urge to vomit. Nausea and vomiting are preventable, treatable side effects. They are common in patients receiving chemotherapy for cancer and in some patients receiving radiation therapy. Many patients with cancer say they fear nausea and vomiting more than any other side effects of treatment, but nausea and vomiting are preventable with medication. When it is minor and treated quickly, nausea and vomiting can be quite uncomfortable but cause no serious problems. Persistent vomiting can cause dehydration, electrolyte imbalance, weight loss, depression, and avoidance of chemotherapy.
Neutropenia. Neutropenia is an abnormally low level of neutrophils, a type of white blood cell. All white blood cells help the body fight infection. Neutrophils fight infection by destroying bacteria. Patients who have neutropenia are at increased risk for developing serious bacterial infections because there are not enough neutrophils to destroy harmful bacteria. Neutropenia occurs in about 50% of patients receiving chemotherapy and is common in patients with leukemia.
Skin problems. The skin is an organ system that contains many nerves. Because of this, skin problems can be very painful. Because the skin is on the outside of the body and visible to others, many patients find coping with skin problems especially difficult. Because the skin protects the inside of the body from infection, skin problems can often lead to other serious problems. As with other side effects, prevention or early treatment is best. In other cases, treatment and wound care can often improve pain and quality of life. Skin problems can have many different causes, including chemotherapy leaking out of the intravenous (IV) tube, which can cause pain or burning; peeling or burned skin caused by radiation therapy; pressure ulcers (bed sores) caused by constant pressure on one area of the body; and pruritus (itching) in patients with cancer, most often caused by leukemia, lymphoma, myeloma, or other cancers.
Thrombocytopenia. Thrombocytopenia is an unusually low level of platelets (thrombocytes) in the blood. Platelets are the blood cells that stop bleeding by plugging damaged blood vessels and helping the blood to clot. Patients with low levels of platelets bleed more easily and are prone to bruising. Certain types of chemotherapy can damage the bone marrow so that it does not make enough platelets. Thrombocytopenia caused by chemotherapy is usually temporary. Other medications used to treat cancer may also lower the number of platelets. In rare instances, a patient’s body can make antibodies to the platelets, lowering the number of platelets.
Regular communication with your doctor is important for making informed decisions about your health care. Consider asking the following questions of your doctor:
What is the exact type of leukemia that I have?
What is the progression (development) of this disease? Is it possible to stop or slow the growth of the cancer? Is it curable?
How can my symptoms be controlled?
What are my treatment options?
What clinical trials are open to me?
What treatment do you recommend? Why?
What type of side effects from treatment can I expect both in the short term and in the long term?
How can I keep myself as healthy as possible during and after treatment?
What follow-up tests will I need, and how often will I need them?
Research for B-cell leukemia is ongoing. The following treatments may still be under investigation in clinical trials and may not be approved or available at this current time. Always discuss all diagnostic and treatment options with your doctor.
New therapies are being tested in clinical trials, including those that use angiogenesis inhibitors and monoclonal antibodies. Angiogenesis inhibitors prevent new blood vessel formation and starve the tumor of oxygen and nutrients. Monoclonal antibodies target proteins that are present in unusually large amounts on the surface of these cells. BL22 and LMB-2 are monoclonal antibodies that are linked to toxins; they 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 whose cancer is no longer responding to other treatments.
American Society for Blood and Marrow Transplantation
85 W. Algonquin Rd., Ste. 550
Arlington Heights, IL 60005
Phone: 847-427-0224 www.asbmt.org
Blood and Marrow Transplant Information Network
2310 Skokie Valley Rd., Ste. 104
Highland Park, IL 60035
Toll Free: 888-597-7674
Phone: 847-433-3313 www.bmtinfonet.org
Leukemia Research Foundation
2700 Patriot Blvd., Ste. 100
Glenview, IL 60026-8021
Toll Free: 888-558-5385
Phone: 847-424-0600 www.leukemia-research.org
National Bone Marrow Transplant Link
20411 W 12 Mile Rd., Ste. 108
Southfield, MI 48076
Toll Free: 800-LINK-BMT (800-546-5268)
Phone: 248-358-1886 www.nbmtlink.org
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 studies 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:
People who have received certain chemotherapy or radiation therapy to the lungs may develop lung damage.
Heart damage in the form of a weakened heart muscle may occur in people who have received a higher dose of doxorubicin (Adriamycin, Rubex) or radiation therapy to the chest.
Infertility or premature menopause can occur in people who have received high-dose cyclophosphamide (Cytoxan, Neosar) or other chemotherapy.
A secondary leukemia or a secondary cancer (a cancer that develops because of the treatment for another type of cancer) is more common in people who were treated with chemotherapy and radiation therapy. Specifically, people with CLL are at higher risk for developing skin cancer and should protect their skin when in the sun.
People recovering from B-cell leukemia are encouraged to follow established guidelines for good health, such as maintaining a healthy weight, 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 Healthy Living After Cancer.
Doctors and scientists are always looking for better ways to treat patients with B-cell leukemia. A clinical trial is a way to test a new treatment in order to prove that it is safe, effective, and possibly better than a standard treatment. Patients who participate in clinical trials are among the first to receive new treatments, such as new chemotherapy before they are widely available. However, there is no guarantee that the new treatment will be safe, effective, or better than a standard treatment.
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 finding new drugs and other therapies is 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.
To join a clinical trial, patients must complete a learning process known as informed consent. During informed consent, the doctor should list all of the patient’s options, so 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 cancer clinical trials.
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