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Table of Contents General Information About Hairy Cell Leukemia
Stages of Hairy Cell Leukemia
Relapsed or Refractory Hairy Cell Leukemia
Treatment Option Overview
Treatment Options for Hairy Cell Leukemia
To Learn More About Hairy Cell Leukemia
Get More Information From NCI
Changes to This Summary (08/01/2008)
About PDQ
General Information About Hairy Cell Leukemia
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Hairy cell leukemia is a type of cancer in which
the bone
marrow makes too many lymphocytes (a type of white blood cell).
Hairy cell leukemia is
a cancer of the blood and bone marrow. This rare type of leukemia gets worse slowly or does not get worse at all. The disease is called hairy cell leukemia
because the leukemia cells look "hairy" when viewed
under a microscope.
Normally,
the bone marrow makes blood stem cells (immature cells) that develop into mature blood cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell.
The myeloid stem cell develops into one of three types of mature blood cells:
The lymphoid stem cell develops into a lymphoblast cell and then into one of three types of lymphocytes (white blood cells):
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| Blood cell development. A blood stem cell goes through several steps to become a red blood cell, platelet, or white blood cell. |
In hairy cell leukemia, too many blood stem cells develop into lymphocytes. These lymphocytes are abnormal and do not become healthy white blood cells. They may also be called leukemic cells. The leukemic cells can build up in the blood and bone marrow so there is less room for healthy white blood cells, red blood cells, and platelets. This may cause infection, anemia, and easy bleeding. Some of the leukemia cells may collect in the spleen and cause it to swell.
This summary is about hairy cell leukemia.
See the following PDQ summaries for information about other types of leukemia:
Gender and age may affect the risk of developing hairy cell leukemia.
Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. People who think they may be at risk should discuss this with their doctor. The cause of hairy cell leukemia is unknown. It occurs more often in older men.
Possible signs of hairy cell leukemia include tiredness, infections, and pain below the ribs.
These and other symptoms may be caused by hairy cell leukemia. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following
problems occur:
- Weakness or feeling tired.
- Fever or frequent infections.
- Easy bruising or bleeding.
- Shortness of breath.
- Weight loss for no known reason.
- Pain or a feeling of fullness below the ribs.
- Painless lumps in the neck, underarm, stomach, or groin.
Tests that examine the blood and bone marrow are used to detect
(find) and diagnose hairy cell leukemia.
The following tests and procedures may be used:
- Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as a swollen spleen, lumps, or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
- Complete blood
count (CBC): A procedure in which a sample of blood is drawn and
checked for the following:
- The number of red blood cells, white blood cells, and
platelets.
- The amount of hemoglobin (the protein that carries oxygen) in
the red blood cells.
- The portion of the sample made up of red blood
cells.
| Enlarge | | | | Complete blood count (CBC). Blood is collected by inserting a needle into a vein and allowing the blood to flow into a tube. The blood sample is sent to the laboratory and the red blood cells, white blood cells, and platelets are counted. The CBC is used to test for, diagnose, and monitor many different conditions. |
- Peripheral blood smear: A procedure in which a sample of blood is checked for cells that look "hairy," the number and kinds of white blood cells, the number of platelets, and changes in the shape of blood cells.
- Bone marrow aspiration and biopsy: The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow, blood, and bone under a microscope to look for signs of cancer.
| Enlarge | | | | Bone marrow aspiration and biopsy. After a small area of skin is numbed, a Jamshidi needle (a long, hollow needle) is inserted into the patient’s hip bone. Samples of blood, bone, and bone marrow are removed for examination under a microscope. |
- Immunophenotyping: A test in which the cells in a sample of blood or bone marrow are looked at under a microscope to check the pattern of proteins that are on the surface of the cells. Hairy cells have a certain pattern.
- CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. A CT scan of the abdomen may be done to check for swollen lymph nodes or a swollen spleen.
Certain factors affect treatment options and prognosis (chance
of recovery).
The treatment options may depend on the following:
- The number of hairy (leukemia) cells and healthy blood cells in the blood and bone marrow.
- Whether the spleen is swollen.
- Whether there are symptoms of leukemia, such as infection.
- Whether the leukemia has recurred (come back) after previous treatment.
The prognosis (chance of recovery) depends on the following:
- Whether the hairy cell leukemia does not grow or grows so slowly it does not need treatment.
- Whether the hairy cell leukemia responds to treatment.
Treatment often results in a long-lasting remission (a period during which some or all of the signs and symptoms of the leukemia are gone). If the leukemia returns after it has been in remission, retreatment often causes another remission.
Back to Top Stages of Hairy Cell Leukemia
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There is no standard staging system for hairy cell leukemia.
Staging is the process
used to find out how far the cancer has spread. Groups are used in place of stages for hairy cell leukemia. The
disease is grouped as untreated, progressive, or refractory.
Untreated hairy cell leukemia
The hairy cell leukemia is newly diagnosed and has not been treated except to relieve symptoms such as weight loss and infections. In untreated hairy cell leukemia, some or all of the following conditions occur:
Progressive hairy cell leukemia
In progressive hairy cell leukemia, the leukemia has been treated with either chemotherapy or splenectomy (removal of the spleen) and one or both of the following conditions occur:
There are three ways that cancer spreads in the body.
When cancer cells spread outside the blood, a solid tumor may form. This process is called metastasis.
The three ways that cancer cells spread in the body are:
- Through the blood. Cancer cells travel through the blood, invade solid tissues in the body, such as the brain or heart, and form a solid tumor.
- Through the lymph system. Cancer cells invade the lymph system, travel through the lymph vessels, and form a solid tumor in other parts of the body.
- Through solid tissue. Cancer cells that have formed a solid tumor spread to tissues in the surrounding area.
The new (metastatic) tumor is the same type of cancer as the primary cancer. For example, if leukemia cells spread to the brain, the cancer cells in the brain are actually leukemia cells. The disease is metastatic leukemia, not brain cancer.
Back to Top Relapsed or Refractory Hairy Cell Leukemia
Relapsed hairy cell leukemia has come back after treatment. Refractory hairy cell leukemia has not responded to treatment.
Back to Top Treatment Option Overview
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There are different types of treatment for patients with hairy
cell leukemia.
Different types of treatment are available for patients with hairy cell leukemia. Some treatments
are standard (the currently used treatment), and some are being tested in clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with cancer.
When clinical trials show that a new treatment is better than the
standard treatment, the new
treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Four types of standard treatment are used:
Watchful waiting
Watchful waiting is closely monitoring a patient's condition, without giving any treatment until symptoms appear or change.
Chemotherapy
Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the spinal column, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Cladribine and pentostatin are anticancer drugs commonly used to treat hairy cell leukemia. These drugs may increase the risk of developing other types of cancer, especially Hodgkin lymphoma and non-Hodgkin lymphoma. Long-term follow up for second cancers is very important.
Biologic therapy
Biologic
therapy is a cancer treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Interferon alfa is a biologic agent commonly used to treat hairy cell leukemia. For relapsed or refractory patients, a biologic agent called rituximab may be used.
Surgery
Splenectomy is a surgical procedure to remove the spleen.
New types of treatment are being tested in clinical trials.
This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI Web site.
Stem cell transplant
Stem cell transplant is a method of giving chemotherapy and replacing blood-forming cells destroyed by the cancer or cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of a brother or sister and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body’s blood cells.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.
Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.
Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.
Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's clinical trials database.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. This is sometimes called re-staging.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
Back to Top Treatment Options for Hairy Cell Leukemia
A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.
Untreated Hairy Cell Leukemia
If the patient's blood cell counts are not too low and there are no symptoms, treatment may not be needed and the patient is carefully watched for changes in his or her condition. If blood cell counts become too low or symptoms appear, initial treatment may include the following:
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with untreated hairy cell leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
Progressive Hairy Cell Leukemia
Treatment for progressive hairy cell leukemia may include the
following:
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with progressive hairy cell leukemia, initial treatment. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
Relapsed or Refractory Hairy Cell Leukemia
Treatment of relapsed or refractory hairy cell leukemia may include the
following:
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with refractory hairy cell leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
Back to Top To Learn More About Hairy Cell Leukemia
For more information from the National Cancer Institute about hairy cell leukemia, see the following:
For general cancer information and other resources from the National Cancer Institute, see the following:
Back to Top Get More Information From NCI
Call 1-800-4-CANCER
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.
Chat online
The NCI's LiveHelp® online chat service provides Internet users with the ability to chat online with an Information Specialist. The service is available from 9:00 a.m. to 11:00 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 to us
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
Search the NCI Web site
The NCI 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. For a quick search, use our “Best Bets” search box in the upper right hand corner of each Web page. The results that are most closely related to your search term will be listed as Best Bets at the top of the list of search results.
There are also many other places to get materials and information about cancer treatment and services. Hospitals in your area may have information about local and regional agencies that have information on finances, getting to and from treatment, receiving care at home, and dealing with problems related to cancer treatment.
Find 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.
Back to Top Changes to This Summary (08/01/2008)
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Text describing the way cancer spreads in the body was added to the Stages section.
Back to Top About PDQ
PDQ is a comprehensive cancer database available on NCI's Web site.
PDQ is the National Cancer Institute's (NCI's) comprehensive cancer information database. Most of the information contained in PDQ is available online at 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.
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 the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Listings of clinical trials are included in PDQ and are available online at NCI's Web site. 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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