National Cancer Institute


Posted Date: Oct 23, 2002

TABLE OF CONTENTS


Description

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What is chronic lymphocytic leukemia?

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Chronic lymphocytic leukemia (CLL) is a disease in which too manyinfection-fighting white blood cells called lymphocytes are found in the body. Lymphocytes are made in the bone marrow and by other organs of the lymphsystem. The bone marrow is the spongy tissue inside the large bones in thebody. The bone marrow makes red blood cells (which carry oxygen and othermaterials to all tissues of the body), white blood cells (which fightinfection), and platelets (which make the blood clot). Normally, bone marrowcells called blasts develop (mature) into several different types of bloodcells that have specific jobs to do in the body.

The lymph system is made up of thin tubes that branch, like blood vessels, intoall parts of the body. Lymph vessels carry lymph, a colorless, watery fluidthat contains lymphocytes. Along the network of vessels are groups of small,bean-shaped organs called lymph nodes. Clusters of lymph nodes are found inthe underarm, pelvis, neck, and abdomen. The spleen (an organ in the upperabdomen that makes lymphocytes and filters old blood cells from the blood), thethymus (a small organ beneath the breastbone), and the tonsils (an organ in thethroat) are also part of the lymph system.

Lymphocytes fight infection by making substances called antibodies, whichattack germs and other harmful things in the body. In CLL, the developinglymphocytes do not mature correctly and too many are made. The lymphocytesmay look normal, but they cannot fight infection as well as they should. These immature lymphocytes are then found in the blood and the bone marrow. They also collect in the lymph tissues and make them swell. Lymphocytes maycrowd out other blood cells in the blood and bone marrow. Anemia may developif the bone marrow cannot make enough red blood cells to carry oxygen. Ifthe bone marrow cannot make enough platelets to make the blood clot normally,bleeding or bruising may occur easily.

Leukemia can be acute (progressing quickly with many immature cells) orchronic (progressing slowly with more mature, normal-looking cells). Chroniclymphocytic leukemia progresses slowly and usually occurs in people 60 yearsof age or older. In the first stages of the disease there are often nosymptoms. As time goes on, more and more lymphocytes are made and symptomsbegin to appear. A doctor should be seen if the lymph nodes swell, thespleen or liver becomes larger than normal, a feeling of fatigue persists, orbleeding occurs easily.

If there are symptoms, a doctor will do a physical examination and may orderblood tests to count the number of each of the different kinds of blood cells. More blood tests may be done if the results of the blood tests are not normal. The doctor also may do a bone marrow biopsy. During this test, a needle isinserted into a bone and a small amount of bone marrow is taken out and lookedat under the microscope. The doctor can then tell what kind of leukemia thepatient has and plan the best treatment.

The chance of recovery (prognosis) depends on the stage of the disease, and thepatient's age and general health.

(Refer to the PDQ summaries on Adult Acute Lymphoblastic Leukemia Treatment;Childhood Acute Lymphoblastic Leukemia Treatment; Adult Acute Myeloid LeukemiaTreatment; Childhood Acute Myeloid Leukemia Treatment; Chronic MyelogenousLeukemia Treatment; and Hairy Cell Leukemia Treatment for more information.)


Stage Explanation

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Stages of chronic lymphocytic leukemia

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Once chronic lymphocytic leukemia has been found (diagnosed), more tests may bedone to find out if leukemia cells have spread to other parts of the body. This is called staging. A doctor needs to know the stage of the disease toplan treatment. The following stages are used for chronic lymphocyticleukemia:


Stage 0

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There are too many lymphocytes in the blood, but there are usually no othersymptoms of leukemia. Lymph nodes and the spleen and liver are not swollen andthe number of red blood cells and platelets is normal.


Stage I

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There are too many lymphocytes in the blood and lymph nodes are swollen. Thespleen and liver are not swollen and the number of red blood cells and platelets isnormal.


Stage II

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There are too many lymphocytes in the blood and lymph nodes and the liver andspleen are swollen.


Stage III

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There are too many lymphocytes in the blood and there are too few red bloodcells (anemia). Lymph nodes and the liver or spleen may be swollen.


Stage IV

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There are too many lymphocytes in the blood and too few platelets, which makeit hard for the blood to clot. The lymph nodes, liver, or spleen may beswollen and there may be too few red blood cells (anemia).


Refractory

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Refractory means that the leukemia does not respond to treatment.


Treatment Option Overview

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How chronic lymphocytic leukemia is treated

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There are treatments for all patients with chronic lymphocytic leukemia. Threekinds of treatment are used:

  • Chemotherapy (using drugs to kill cancer cells).
  • Radiation therapy (using high-dose x-rays or other high-energy rays to killcancer cells).
  • Treatment for complications of the leukemia, such as infection.

The use of biological therapy (using the body's immune system to fight cancer)is being tested in clinical trials. Surgery may be used in certain cases.

Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be taken bypill, or it may be put into the body by a needle in the vein or muscle. Chemotherapy is called a systemic treatment because the drug enters thebloodstream, travels through the body, and can kill cancer cells throughout thebody.

Radiation therapy uses x-rays or other high-energy rays to kill cancer cellsand shrink tumors. Radiation for CLL usually comes from a machine outside thebody (external radiation therapy).

If the spleen is swollen, a doctor may take out the spleen in an operationcalled a splenectomy. This is only done in rare cases.

Biological therapy tries to get the body to fight cancer. It uses materialsmade by the body or made in a laboratory to boost, direct, or restore thebody's natural defenses against disease. Biological therapy is sometimescalled biological response modifier (BRM) therapy or immunotherapy.

Because infection often occurs in patients with CLL, a special substance calledimmunoglobulin, which contains antibodies, may be given to prevent infections.

Sometimes a special machine is used to filter the blood to take out extralymphocytes. This is called leukapheresis.

Bone marrow transplantation is used to replace the bone marrow with healthybone marrow. First, all of the bone marrow in the body is destroyed with highdoses of chemotherapy with or without radiation therapy. Healthy marrow isthen taken from another person (a donor) whose tissue is the same as or almostthe same as the patient's. The donor may be a twin (the best match), a brotheror sister, or another person not related. The healthy marrow from the donor isgiven to the patient through a needle in the vein, and the marrow replaces themarrow that was destroyed. A bone marrow transplant using marrow from arelative or person not related to the patient is called an allogeneic bonemarrow transplant.

Another type of bone marrow transplant, called autologous bone marrowtransplant, is being studied in clinical trials. To do this type oftransplant, bone marrow is taken from the patient and treated with drugs tokill any cancer cells. The marrow is frozen to save it. Next, the patient isgiven high-dose chemotherapy with or without radiation therapy to destroy allof the remaining marrow. The frozen marrow that was saved is then thawed andgiven back to the patient through a needle in a vein to replace the marrow thatwas destroyed.


Treatment by stage

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Treatment of chronic lymphocytic leukemia depends on the stage of the disease,and the patient's age and overall health.

Standard treatment may be considered because of its effectiveness in patientsin past studies, or participation in a clinical trial may be considered. Mostpatients with chronic lymphocytic leukemia are not cured with standard therapyand some standard treatments may have more side effects than are desired. Forthese reasons, clinical trials are designed to find better ways to treat cancerpatients and are based on the most up-to-date information. Clinical trials areon going in most parts of the country for most stages of chronic lymphocyticleukemia. To know more about clinical trials, call the Cancer InformationService at 1-800-4-CANCER (1-800-422-6237); TTY at 1-800-332-8615.


Stage 0 Chronic Lymphocytic Leukemia

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If the patient has stage 0 CLL, treatment may not be needed or chemotherapy maybe given. A doctor will follow the patient closely so treatment can be startedif the leukemia gets worse.


Stage I Chronic Lymphocytic Leukemia

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Treatment may be one of the following:

  1. If there are no symptoms, no treatment may be needed. A doctor willfollow the patient closely so treatment can be started if the leukemiagets worse.
  2. Chemotherapy with or without steroids.
  3. Other chemotherapy drugs.
  4. External radiation therapy to swollen lymph nodes.
  5. Combination chemotherapy.
  6. A clinical trial evaluating monoclonal antibodies.
  7. A clinical trial evaluating bone marrow or peripheral stem celltransplantation.


Stage II Chronic Lymphocytic Leukemia

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Treatment may be one of the following:

  1. If there are few or no symptoms, no treatment may be needed. A doctorwill follow the patient closely so treatment can be started if theleukemia gets worse.
  2. Chemotherapy with or without steroids.
  3. Other chemotherapy drugs.
  4. Combination chemotherapy.
  5. External radiation therapy to swollen lymph nodes.
  6. External radiation therapy to the spleen to reduce symptoms caused by the enlargement of the spleen.
  7. A clinical trial evaluating monoclonal antibodies.
  8. A clinical trial evaluating bone marrow or peripheral stem celltransplantation.


Stage III Chronic Lymphocytic Leukemia

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Treatment may be one of the following:

  1. If there are few or no symptoms, no treatment may be needed. A doctorwill follow the patient closely so treatment can be started if theleukemia gets worse.
  2. Chemotherapy with or without steroids.
  3. Other chemotherapy drugs.
  4. Combination chemotherapy.
  5. Surgery to remove the spleen or external radiation therapy to the spleento reduce symptoms caused by the enlargement of the spleen.
  6. A clinical trial evaluating monoclonal antibodies.
  7. A clinical trial evaluating bone marrow or peripheral stem celltransplantation.


Stage IV Chronic Lymphocytic Leukemia

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Treatment may be one of the following:

  1. If there are few or no symptoms, no treatment may be needed. A doctorwill follow the patient closely so treatment can be started if theleukemia gets worse.
  2. Chemotherapy with or without steroids.
  3. Other chemotherapy drugs.
  4. Combination chemotherapy.
  5. Surgery to remove the spleen or external radiation therapy to the spleento reduce symptoms caused by the enlargement of the spleen.
  6. A clinical trial evaluating monoclonal antibodies.
  7. A clinical trial evaluating bone marrow or peripheral stem celltransplantation.


Refractory Chronic Lymphocytic Leukemia

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Treatment depends on many factors; patients may wish to consider entering aclinical trial of new chemotherapy drugs and bone marrow or peripheral stemcell transplantation.


To Learn More

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Call

For more information, U.S. residents may call the National Cancer Institute's (NCI's) Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237) Monday through Friday from 9:00 a.m. to 4:30 p.m. Deaf and hard-of-hearing callers with TTY equipment may call 1-800-332-8615. The call is free and a trained Cancer Information Specialist is available to answer your questions.

Web sites and Organizations

The NCI's Cancer.gov Web site provides online access to information on cancer, clinical trials, and other Web sites and organizations that offer support and resources for cancer patients and their families. There are also many other places where people can get materials and information about cancer treatment and services. Local hospitals may have information on local and regional agencies that offer information about finances, getting to and from treatment, receiving care at home, and dealing with problems associated with cancer treatment.

Publications

The NCI has booklets and other materials for patients, health professionals, and the public. These publications discuss types of cancer, methods of cancer treatment, coping with cancer, and clinical trials. Some publications provide information on tests for cancer, cancer causes and prevention, cancer statistics, and NCI research activities. NCI materials on these and other topics may be ordered online or printed directly from the NCI Publications Locator. These materials can also be ordered by telephone from the Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.

LiveHelp

The NCI's LiveHelp service, a program available on several of the Institute's Web sites, provides Internet users with the ability to chat online with an Information Specialist. The service is available from 9:00 a.m. to 10:00 p.m. Eastern time, Monday through Friday. Information Specialists can help Internet users find information on NCI Web sites and answer questions about cancer.

Write

For more information from the NCI, please write to this address:

  • NCI Public Inquiries Office
  • Suite 3036A
  • 6116 Executive Boulevard, MSC8322
  • Bethesda, MD 20892-8322


About PDQ

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PDQ is a comprehensive cancer database available on Cancer.gov.

PDQ is the National Cancer Institute's (NCI's) comprehensive cancer information database. Most of the information contained in PDQ is available online at Cancer.gov, the NCI's Web site. PDQ is provided as a service of the NCI. The NCI is part of the National Institutes of Health, the federal government's focal point for biomedical research.

PDQ contains cancer information summaries.

The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries are available in two versions. The health professional versions provide detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions provide current and accurate cancer information.

The PDQ cancer information summaries are developed by cancer experts and reviewed regularly.

Editorial Boards made up of experts in oncology and related specialties are responsible for writing and maintaining the cancer information summaries. The summaries are reviewed regularly and changes are made as new information becomes available. The date on each summary ("Date Last Modified") indicates the time of the most recent change.

PDQ also contains information on clinical trials.

Before starting treatment, patients may want to think about taking part in a clinical trial. A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about new treatments, the risks involved, and how well they do or do not work. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard."

Listings of clinical trials are included in PDQ and are available online at Cancer.gov. Descriptions of the trials are available in health professional and patient versions. Many cancer doctors who take part in clinical trials are also listed in PDQ. For more information, call the Cancer Information Service 1-800-4-CANCER (1-800-422-6237); TTY at 1-800-332-8615.

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News
Appears to increase risk after liver transplant in younger patients, those with C2 monitoring

Jul 1, 2010 - Immunosuppressive treatment with cyclosporine A, rather than tacrolimus, with dose level monitoring two hours post-dosing or in patients age 50 or younger appears to have a significant association with the development of de novo cancer after liver transplantation, according to research published in the July issue of Liver Transplantation.



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