Leukemia is a cancer of the blood cells. Blood cells (including red cells that carry oxygen, white cells to fight infection, and platelets that cause blood to clot) are produced in the bone marrow, the spongy tissue inside the larger bones in the body. Abnormalities in the bone marrow cells can cause the overproduction or underproduction of certain 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 also other, less common types of leukemia, but they are generally subcategories of one of the four main categories. This section focuses on different types of chronic T-cell lymphocytic leukemia, a subtype of chronic lymphocytic leukemia (CLL). A T cell is a type of white blood cell that directly participates in the immune system defenses.
Subtypes of T-cell leukemia
Large granular lymphocytic leukemia (LGLL). LGLL is a slow-growing leukemia of the T cells and is more common in women than in men. The cause of LGLL is unknown, although approximately 30% of people with LGLL also have rheumatoid arthritis (a chronic, progressive disease in which inflammatory changes occur throughout the connective tissues of the body, mostly in the joints of the hands, feet, wrists, knees, hips, or shoulders).
T-cell prolymphocytic leukemia (T-PLL). T-PLL is an aggressive subtype of CLL that has only been defined recently. It occurs more often in older men, but women may also be affected. It can affect the skin, but in a different way than Sezary syndrome (see below).
Adult T-cell leukemia/lymphoma (ATLL). ATLL has four clinical subtypes. Depending on the clinical features, it is subclassified as smoldering, chronic, acute, or adult T-cell lymphoma (cancer of the lymph system). The acute and the adult T-cell lymphoma subtypes are rapidly progressing diseases. ATLL is caused by a retrovirus called the human T-cell leukemia virus (HTLV1).
Sezary syndrome. Sezary syndrome is a form of mycosis fungoides, a T-cell lymphoma that occurs in the skin. Sezary syndrome is usually slow-growing and takes years to develop from mycosis fungoides, which is limited to the skin. Sezary syndrome is generally diagnosed when large numbers of the lymphoma cells are found in the blood, often in association with erythroderma (reddening of the skin).
Statistics
In 2009, an estimated 44,790 people of all ages (25,630 men and 19,160 women) in the United States will be diagnosed with leukemia. Of these, an estimated 15,490 people (9,200 men and 6,290 women) will be diagnosed with CLL. T-cell leukemia is rare and the number of people diagnosed each year is much lower.
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 2009.
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.
In general, leukemia can be caused by a hereditary genetic mutation or environmental factors (including smoking, chemical, or radiation exposure). However, most cases of leukemia have unknown causes.
The following risk factor can raise a person’s risk of developing T-cell leukemia:
Race/Ethnicity. ATLL is more common in people of Japanese, West Indian, and African American descent.
People with T-cell leukemia may experience the following symptoms. Sometimes, people with T-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.
Recurrent infections, due to low numbers of neutrophils (infection-fighting white blood cells)
Bleeding or bruising easily
Unexplained tiredness (fatigue)
Persistent, unexplained abdominal pain on the left side, due to an enlarged spleen (LGLL, T-PLL)
Enlarged lymph nodes (T-PLL, ATLL)
Rash or skin lesions (T-PLL, ATLL, Sezary syndrome)
Frequent urination and/or constipation, due to high levels of calcium in the blood (ATLL)
Doctors use many blood and bone marrow tests to diagnose leukemia and to determine 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/or 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 suspected
Severity of symptoms
Previous test results
In addition to a physical examination, the following tests may be used to diagnose T-cell leukemia:
Blood tests. A doctor will do a routine blood test called a complete blood count (CBC) to measure the counts of different types of cells in the blood. If the blood contains high levels of white blood cells, T-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 (proteins that can induce an immune response), on the surface of the leukemic cells. Immunophenotyping allows the doctor to confirm the exact type of leukemia.
Cytogenetics. Cytogenetics is the examination of the leukemic 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 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.
Skin biopsy. A skin biopsy is a procedure in which a sample of skin tissue is removed and examined under a microscope to look for the presence and type of T cells in the skin.
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, there is no standard staging system for LGLL, T-PLL, ATLL, or Sezary syndrome.
The treatment of T-cell leukemia depends on the symptoms produced by 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.
This section outlines treatments that are the standard of care (the best treatments available) for this specific type of cancer. Patients are also encouraged to consider clinical trials as a treatment option when making treatment plan decisions. A clinical trial is a research study to test a new treatment to prove it is safe, effective, and possibly better than standard treatment. Your doctor can help you review all treatment options. For more information, visit the Clinical Trials section.
General treatment options for T-cell leukemia are described below, followed by an outline of the treatment options by each subtype.
Active surveillance/watch and wait
This approach means that the doctor is closely monitoring the disease, and active treatment begins only when the leukemia shows signs of advancing. It may also be called active surveillance or watchful waiting. Patients are monitored using blood and other tests at regularly scheduled checkups to track blood cell counts and look for other symptoms that the leukemia is progressing. Studies have shown that, in people with certain disease characteristics, no harm comes from the watch-and-wait approach versus offering early treatment. Treatment begins when people develop signs that the disease is worsening, such as increasing fatigue, night sweats, enlarged lymph nodes, or falling blood cell counts. People with leukemia are encouraged to talk with their doctors about whether their symptoms need treatment, and to consider the benefits of treatment versus side effects that might result.
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.
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 through Cancer.Net’s Drug Information Resources, which provides links to searchable drug databases.
Radiation therapy
Radiation therapy is the use of x-rays or other high-energy particles to kill cancer cells. To treat leukemia, radiation therapy usually comes from a machine outside the body and is called external-beam radiation therapy.
Side effects from radiation therapy include fatigue, mild skin reactions, upset stomach, and loose bowel movements. Most side effects go away soon after treatment is finished.
Surgery
Surgery to remove the spleen (splenectomy), which also produces white blood cells, may occasionally be performed.
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 blood stem cells are typically what is 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.
Stem cell transplantation is not a common treatment option for people with T-cell leukemia, because it is not a consistently effective therapy for this disease and because many patients with this disease are older and the risks of 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).
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; matches can be made by searching a computer registry. 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 cancer cells in the marrow, blood, and other parts of the body and have 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 transplant types, 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.
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, in that the donor cells can recognize the cancer 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 preventative 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 be cancerous.
Immunotherapy or biologic therapy is designed to boost the body's natural defenses to fight the cancer. It uses materials either made by the body or in a laboratory to bolster, target, or restore immune system function. Of these, recombinant interferon alpha (Alferon N, Roferon-A, Intron A) has been found to be an effective therapy in ATLL. Interferon is a natural protein present in the body that stimulates immunity.
Monoclonal antibodies
Monoclonal antibodies are antibodies directed against specific proteins on the leukemic cell surface. When the antibody attaches to their antigen, some leukemic cells die.
Treatments specific to T-cell leukemia type
LGLL. LGLL sometimes requires no treatment beyond watch-and-wait during its early stages, but treatment may begin once symptoms develop. Sometimes, infections requiring treatment with antibiotics occur because of the limited numbers of neutrophils. When treatment for the disease does become necessary, LGLL is treated with immunosuppressant drugs, such as cyclosporine (multiple brand names), cyclophosphamide (Clafen, Cytoxan, Neosar), or low-dose methotrexate (multiple brand names). Treatment with oral cyclosporine, a drug that suppresses the immune system, can be effective in some people in whom low levels of neutrophils or platelets represent the major problem. Treatment with growth factors (granulocyte colony stimulating factors, Neupogen) which can stimulate the growth of neutrophils, are sometimes used when infections, due to low neutrophil counts, become a problem. Treatment with combination chemotherapy (the use of more than one drug) is occasionally used if the disease is advancing rapidly. Combination chemotherapy is similar to that used in aggressive lymphoma; for more information, see the Cancer.Net Guide to Non-Hodgkin Lymphoma.
T-PLL. T-PLL may be treated with drugs that include fludarabine, chlorambucil (Ambochlorin, Leukeran), cyclophosphamide, doxorubicin hydrochloride (Adriamycin, Rubex), vincristine (Oncovin), pentostatin (Nipent), and prednisone. Alemtuzumab (Campath) is a monoclonal antibody directed against a protein called CD52 which is found on the surface of certain T cells and has shown promising results in some patients with T-PLL.
Adult T-cell leukemia/lymphoma (ATLL). ATLL may be treated with zidovudine (Retrovir) and recombinant interferon alpha if it is in the chronic or acute phase. Zidovudine is an antiviral therapy used in the treatment of human immunodeficiency virus (HIV). This therapy aims at strengthening the immune system and treating the human T-cell leukemia virus (HTLV). The lymphoma phase is best treated with combination chemotherapy.
Sezary syndrome. Sezary syndrome may be treated using therapies focused on the skin and systemic whole-body therapies. Skin therapies include topical agents (skin creams); phototherapy (the use of light to kill cancer cells); and radiation therapy, including total skin electron-beam radiation therapy, which can treat the entire body. Systemic therapies for Sezary syndrome include chemotherapy, oral bexarotene (Targretin) (a drug that is similar to vitamin A), denileukin diftitox (Ontak), alpha interferon, and occasionally stem cell transplantation. Alemtuzumab can also be somewhat effective for treating this disease. Recently, a drug, vorinostat (Zolinza), was approved by the U.S. Food and Drug Administration (FDA) cutaneous T-cell lymphoma not responding to other treatments. The choice of treatment depends on the extent of the disease and other factors.
Doctors and scientists are always looking for better ways to treat patients with T-cell leukemia. A clinical trial is a way to test a new treatment 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 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 T-cell leukemia. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with T-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.
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 do 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 the person’s overall health.
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 if they do happen. Also, be sure to communicate with the doctor about side effects you experience during and after treatment. For more information on the most common side effects of cancer and different treatments, along with ways to prevent or control them, visit Cancer.Net’s section on Managing Side Effects, based on ASCO’s curriculum.
In addition to physical side effects, there may be psychosocial (emotional and social) effects as well. Learn more about the importance of addressing these needs in Cancer.Net’s section on Caring for the Whole Patient.
For more information on late effects or long-term side effects, please read the After Treatment section or talk with your doctor.
After treatment for T-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.
Cardiac damage in the form of a weakened heart muscle may occur in people who have received a higher dose of doxorubicin or radiation therapy to the chest.
Infertility or premature menopause can occur in people who have received high-dose cyclophosphamide or other chemotherapy.
People treated with chemotherapy, and specifically with alemtuzumab, are at higher risk for infection even after the end of therapy.
A secondary leukemia or 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.
People recovering from T-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 Healthy Living After Cancer.
Research for T-cell leukemia is ongoing. The following treatments may still be under investigation in clinical trials and may not be approved or available at this time. Always discuss all diagnostic and treatment options with your doctor.
New therapies are being tested in clinical trials, including new combinations of chemotherapy and biologic therapies, which use the body’s immune system to fight cancer. For ATLL, several new therapies are being explored including bortezomib (Velcade); arsenic trioxide (Trisenox); and daclizumab (Zenapax), an anti-IL2 antibody.
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 T-cell leukemia that I have?
Can you explain my pathology report to me?
How aggressive is my disease? Is it curable?
Is it possible to stop its development?
How can my symptoms be controlled?
What are my treatment options?
What clinical trials are available to me?
What treatment do you recommend? Why?
What are the possible side effects of this treatment, both in the short term and the long term?
How will this treatment affect my daily life? Will I be able to work, exercise, and perform my usual activities?
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?
What support services are available to me? To my family?
Rare Cancer Alliance
1649 N. Pacana Way
Green Valley, AZ 85614 www.rare-cancer.org
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
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
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