|
Table of Contents General Information About Chronic Myeloproliferative Disorders
Chronic Myelogenous Leukemia
Polycythemia Vera
Chronic Idiopathic Myelofibrosis
Essential Thrombocythemia
Chronic Neutrophilic Leukemia
Chronic Eosinophilic Leukemia
Stages of Chronic Myeloproliferative Disorders
Treatment Option Overview
Treatment Options for Chronic Myeloproliferative Disorders
To Learn More About Chronic Myeloproliferative Disorders
Get More Information From NCI
Changes to This Summary (08/01/2008)
About PDQ
General Information About Chronic Myeloproliferative Disorders
| Key Points for This Section
|
|
Myeloproliferative disorders are a group of diseases in which the bone marrow makes too many red blood cells, white blood cells, or platelets.
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 lymphoid stem cell develops into a white blood cell. The myeloid stem cell develops into one of three types of mature blood cells:
|
Enlarge
|
|
|
|
|
| Blood cell development. A blood stem cell goes through several steps to become a red blood cell, platelet, or white blood cell. |
In myeloproliferative disorders too many blood stem cells develop into one or more types of blood cells. The disorders usually get worse slowly as the number of extra blood cells increases.
There are 6 types of chronic myeloproliferative disorders.
The type of myeloproliferative disorder is based on whether too many red blood cells, white blood cells, or platelets are being made. Sometimes the body will make too many of more than one type of blood cell, but usually one type of blood cell is affected more than the others are. Chronic myeloproliferative disorders include the following 6 types:
These types are described below. Chronic myeloproliferative disorders sometimes become acute leukemia, in which too many abnormal white blood cells are made.
Tests that examine the blood and bone marrow are used to detect (find) and diagnose chronic myeloproliferative disorders.
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 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) with differential: A procedure in which a sample of blood is drawn and checked for the following:
- The number of red blood cells and platelets.
- The number and type of white blood cells.
- The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.
- The portion of the blood 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. |
- 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. |
- Cytogenetic analysis: A test in which cells in a sample of blood or bone marrow are viewed under a microscope to look for certain changes in the chromosomes. Certain diseases or disorders may be diagnosed or ruled out based on the chromosomal changes.
Back to Top Chronic Myelogenous Leukemia
Chronic myelogenous leukemia is a disease in which too many white blood cells are made in the bone marrow. See the PDQ summary on Chronic Myelogenous Leukemia Treatment for information on diagnosis, staging, and treatment.
Back to Top Polycythemia Vera
| Key Points for This Section
|
|
|
Polycythemia vera is a disease in which too many red blood cells are made in the bone marrow.
In polycythemia vera, the blood becomes thickened with too many red blood cells. The number of white blood cells and platelets may also increase. These extra blood cells may collect in the spleen and cause it to swell. The increased number of red blood cells or platelets in the blood can cause bleeding problems and make clots form in blood vessels. This can cause serious health problems such as stroke or heart attack. In patients older than 65 years, the risk of stroke and heart attack is higher, and polycythemia vera is more likely to become acute myeloid leukemia or chronic idiopathic myelofibrosis.
Possible signs of polycythemia vera include headaches and a feeling of fullness below the ribs on the left side.
Polycythemia vera often does not cause early symptoms. It is sometimes found during a routine blood test. Symptoms may occur as the number of blood cells increases. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:
- A feeling of pressure or fullness below the ribs on the left side.
- Headaches.
- Double vision or seeing dark or blind spots that come and go.
- Itching all over the body, especially after being in warm or hot water.
- Reddened face that looks like a blush or sunburn.
- Weakness.
- Dizziness.
- Weight loss for no known reason.
Special blood tests are used to diagnose polycythemia vera.
In addition to a complete blood count, other special blood tests are used to diagnose polycythemia vera. These tests include the following:
- Arterial blood gas (ABG) test: A test in which a sample of blood is taken from an artery to measure the amount of oxygen and carbon dioxide in the blood and the pH (acidity) of the blood.
- Serum erythropoietin test: A test in which a sample of blood is checked for the level of erythropoietin (a hormone that stimulates new red blood cells to be made).
- Leukocyte alkaline phosphatase (LAP) test: A test in which a sample of blood is checked to measure the amount of alkaline phosphatase (an enzyme) in leukocytes (white blood cells).
Back to Top Chronic Idiopathic Myelofibrosis
| Key Points for This Section
|
|
|
Chronic idiopathic myelofibrosis is a disease in which abnormal blood cells and fibers build up inside the bone marrow.
The bone marrow is made of tissues that make blood cells (red blood cells, white blood cells, and platelets) and a web of fibers that support the blood-forming tissues. In chronic idiopathic myelofibrosis, large numbers of blood stem cells develop into blood cells that do not mature properly (blasts). The web of fibers inside the bone marrow also becomes very thick (like scar tissue) and slows the blood-forming tissue’s ability to make blood cells. This causes the blood-forming tissues to make fewer and fewer blood cells. In order to make up for the low number of blood cells made in the bone marrow, the liver and spleen begin to make the blood cells.
Possible signs of chronic idiopathic myelofibrosis include pain below the ribs on the left side and feeling very tired.
Chronic idiopathic myelofibrosis often does not cause early symptoms. It is sometimes found during a routine blood test. The following symptoms may be caused by chronic idiopathic myelofibrosis or by other conditions. A doctor should be consulted if any of these problems occur:
- Feeling pain or fullness below the ribs on the left side.
- Feeling full sooner than normal when eating.
- Feeling very tired.
- Shortness of breath.
- Easy bruising or bleeding.
- Petechiae (flat, red, pinpoint spots under the skin that are caused by bleeding).
- Fever.
- Night sweats.
- Weight loss.
A special blood test is used to diagnose chronic idiopathic myelofibrosis.
In addition to a complete blood count, bone marrow aspiration and biopsy, and cytogenetic analysis, a peripheral blood smear is used to diagnose chronic idiopathic myelofibrosis. A peripheral blood smear is a procedure in which a sample of blood is checked for tear drop-shaped red blood cells, the number and kinds of white blood cells, the number of platelets, and the presence of blast cells.
Certain factors affect prognosis (chance of recovery) and treatment options for chronic idiopathic myelofibrosis.
Prognosis (chance of recovery) depends on the following:
- The age of the patient.
- The number of abnormal red blood cells and white blood cells.
- The number of blasts in the blood.
- Whether there are certain changes in the chromosomes.
- Whether the patient has symptoms such as fever, night sweats, or weight loss.
Back to Top Essential Thrombocythemia
| Key Points for This Section
|
|
|
Essential thrombocythemia is a disease in which too many platelets are made in the bone marrow.
Essential thrombocythemia causes an abnormal increase in the number of platelets made in the blood and bone marrow.
Patients with essential thrombocythemia may have no symptoms.
Essential thrombocythemia often does not cause early symptoms. It is sometimes found during a routine blood test. The following symptoms may be caused by essential thrombocytopenia or by other conditions. A doctor should be consulted if any of these problems occur:
- Headache.
- Burning or tingling in the hands or feet.
- Redness and warmth of the hands or feet.
- Vision or hearing problems.
Platelets are sticky. When there are too many platelets, they may clump together and make it hard for the blood to flow. Clots may form in blood vessels and there may also be increased bleeding. These can cause serious health problems such as stroke or heart attack.
Certain factors affect prognosis (chance of recovery) and treatment options for essential thrombocythemia.
Prognosis (chance of recovery) and treatment options depend on the following:
- The age of the patient.
- Whether the patient has symptoms or other problems related to essential thrombocythemia.
Back to Top Chronic Neutrophilic Leukemia
Chronic neutrophilic leukemia is a disease in which too many blood stem cells develop into a type of white blood cell called neutrophils. Neutrophils are infection -fighting blood cells that surround and destroy dead cells and foreign substances (such as bacteria). The spleen and liver may swell because of the extra neutrophils. Chronic neutrophilic leukemia may stay the same or it may progress quickly to acute leukemia.
Back to Top Chronic Eosinophilic Leukemia
| Key Points for This Section
|
|
|
Chronic eosinophilic leukemia is a disease in which too many white blood cells (eosinophils) are made in the bone marrow.
Eosinophils are white blood cells that react to allergens (substances that cause an allergic response) and help fight infections caused by certain parasites. In chronic eosinophilic leukemia, there are too many eosinophils in the blood, bone marrow, and other tissues. Chronic eosinophilic leukemia may stay the same for many years or it may progress quickly to acute leukemia.
Possible signs of chronic eosinophilic leukemia include fever and feeling very tired.
Chronic eosinophilic leukemia may not cause early symptoms. It is sometimes found during a routine blood test. The following symptoms may be caused by chronic eosinophilic leukemia or by other conditions. A doctor should be consulted if any of these problems occur:
- Fever.
- Feeling very tired.
- Cough.
- Swelling under the skin around the eyes and lips, in the throat, or on the hands and feet.
- Muscle pain.
- Itching.
- Diarrhea.
Back to Top Stages of Chronic Myeloproliferative Disorders
| Key Points for This Section
|
|
|
There is no standard staging system for chronic myeloproliferative disorders.
Staging is the process used to find out how far the cancer has
spread. There is no standard staging system for chronic myeloproliferative disorders. Treatment is based on the type of myeloproliferative disorder the patient has. It
is important to know the type in order to plan treatment.
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 Treatment Option Overview
| Key Points for This Section
|
|
|
There are different types of treatment for patients with chronic myeloproliferative disorders.
Different types of treatments are available for patients with chronic myeloproliferative disorders. 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. 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.
Ten 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.
Phlebotomy
Phlebotomy is a procedure in which blood is taken from a vein. A sample of blood may be taken for tests such as a CBC or blood chemistry. Sometimes phlebotomy is used as a treatment and blood is taken from the body to remove extra red blood cells. Phlebotomy is used in this way to treat some chronic myeloproliferative disorders.
Platelet apheresis
Platelet apheresis is a treatment that uses a special machine to remove platelets from the blood. Blood is taken from the patient and put through a blood cell separator where the platelets are removed. The rest of the blood is then returned to the patient’s bloodstream.
Transfusion therapy
Transfusion therapy (blood transfusion) is a method of giving red blood cells, white blood cells, or platelets to replace blood cells destroyed by disease or cancer treatment.
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.
Radiation therapy
Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated.
Radiation therapy to treat myeloproliferative disorders is usually directed at the spleen.
Other drug therapy
Anagrelide therapy is used to reduce the risk of blood clots in patients who have too many platelets in
their blood. Thalidomide and lenalidomide are drugs that prevent blood vessels from growing into areas of tumor cells.
Surgery
Splenectomy (surgery to remove the spleen) may be done if the spleen is enlarged.
Biologic therapy
Biologic therapy is a 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 some chronic myeloproliferative disorders.
High-dose chemotherapy with stem cell transplant
High-dose chemotherapy with stem cell transplant is a method of giving high doses of chemotherapy and replacing blood-forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor 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.
New types of treatment are being tested in clinical trials.
Information about clinical trials is available from the NCI Web site.
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 Chronic Myeloproliferative Disorders
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.
Chronic Myelogenous Leukemia
See the PDQ summary on Chronic Myelogenous Leukemia Treatment for information.
Polycythemia Vera
The purpose of treatment for polycythemia vera is to reduce the number of extra blood cells. Treatment of polycythemia vera may include the following:
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with polycythemia vera. 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.
Chronic Idiopathic Myelofibrosis
Treatment of chronic idiopathic myelofibrosis in patients without symptoms is usually watchful waiting.
Treatment of chronic idiopathic myelofibrosis in patients with symptoms may include the following:
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with chronic idiopathic myelofibrosis. 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.
Essential Thrombocythemia
Treatment of essential thrombocythemia in patients younger than 60 years who have no symptoms and an acceptable platelet count is usually watchful waiting. Treatment of other patients may include the following:
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with essential thrombocythemia. 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.
Chronic Neutrophilic Leukemia
Treatment of chronic neutrophilic 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 chronic neutrophilic 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.
Chronic Eosinophilic Leukemia
Treatment of chronic eosinophilic 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 chronic eosinophilic 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 Chronic Myeloproliferative Disorders
For more information from the National Cancer Institute about chronic myeloproliferative disorders, 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.
Back to Top |
)
. 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.','popup','width=780,height=630,scrollbars=1,resizable=1,menubar=0,location=0,status=0,toolbar=0'))
 is inserted into the patient\'s hip bone. Samples of blood, bone, and bone marrow are removed for examination under a microscope.','popup','width=780,height=630,scrollbars=1,resizable=1,menubar=0,location=0,status=0,toolbar=0'))