Article: Chronic Myeloproliferative Disorders Treatment

Description

What are myeloproliferative disorders?

Myeloproliferative disorders are diseases in which too many of certain types of blood cells are made in the bone marrow. The bone marrow is the spongy tissue inside the large bones in the body. The bone marrow makes red blood cells (which carry oxygen to all the tissues in the body), white blood cells (which fight infection), and platelets (which make the blood clot).

There are six types of myeloproliferative disorders: chronic myelogenous leukemia, polycythemia vera, chronic idiopathic myelofibrosis, essential thrombocythemia, chronic neutrophilic leukemia, and chronic eosinophilic leukemia. Chronic myelogenous leukemia affects the cells that are developing into white blood cells, called granulocytes. (Refer to the PDQ summary on Chronic Myelogenous Leukemia Treatment for more information.)

Polycythemia vera means too many red blood cells are made in the bone marrow and build up in the blood. The spleen (the organ in the upper abdomen that filters the blood to remove old cells) may swell because the extra blood cells collect there. Also, a person may have itching all over the body.

Chronic idiopathic myelofibrosis means red blood cells and certain white blood cells called granulocytes do not mature properly. The red blood cells look like teardrops instead of discs. The spleen may swell and there may be too few mature red blood cells to carry oxygen, causing anemia.

Essential thrombocythemia means the number of platelets in the blood is much higher than normal without any known cause, but other cells in the blood are normal. The extra platelets make it hard for the blood to flow normally.

Chronic neutrophilic leukemia means the number of the principal phagocyte (microbe-eating) cells that combat infection in the blood is much higher than normal without any known cause. The spleen and liver may swell.

Chronic eosinophilic leukemia means the number of white blood cells that participate in allergic reactions and help to fight certain parasitic infections is much higher than normal without any known cause.

If there are symptoms, a doctor will order blood tests to count the numbers of each of the different cells in the blood. If the results of the tests are not normal, more blood tests may be done. The doctor may also do a bone marrow biopsy. During this test, a needle is inserted into a bone to take out some of the marrow. The marrow is then looked at under a microscope. The doctor can then tell what kind of disease the patient has and plan the best treatment.

The chance of recovery (prognosis) depends on the type of myeloproliferative disorder, and the patient's age and general health. The diseases usually vary from person to person, often progressing slowly and requiring little treatment. In some people, the disease may turn into an acute leukemia, in which too many white blood cells are made.

Stage Explanation

Stages of myeloproliferative disorders

There is no staging for these diseases. Treatment depends on the type of myeloproliferative disorder the patient has.

Treatment Option Overview

How myeloproliferative disorders are treated

There are treatments for all patients with myeloproliferative disorders. Usually the diseases cannot be cured, but the symptoms can be controlled and the number of blood cells can be reduced with treatment. Sometimes there are few symptoms and no treatment is needed.

Chemotherapy uses drugs to kill extra blood cells in the body. Chemotherapy may be taken by pill, 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 the bloodstream, travels through the body, and can kill cells throughout the body.

Phlebotomy is taking blood from the body by a needle in a vein. This treatment is used in polycythemia vera to lower the amount of blood in the body.

Sometimes a special machine is used to filter platelets from the blood. This is called plateletpheresis.

Radiation therapy can be used to relieve symptoms. Radiation therapy uses high-energy x-rays to kill cells. Radiation therapy for the myeloproliferative disorders is usually given from a machine outside the body (external-beam radiation therapy). A radioactive drug called P32 can also be given by a needle in a vein to lower the number of red blood cells made by the bone marrow.

Hormones can also be used in certain instances to treat side effects of the disease. In patients with chronic idiopathic myelofibrosis, hormones called glucocorticoids may be given to help the red blood cells live longer. Hormones called androgens are also sometimes used in this disease to make the bone marrow produce more blood cells.

Surgery to remove the spleen (splenectomy) may be done if the spleen is swollen.

Biological therapy is being tested for the treatment of myeloproliferative disorders. Biological therapy tries to get the body to fight disease. It uses materials made by the body or made in a laboratory to boost, direct, or restore the body's natural defenses against disease. Biological therapy is sometimes called biological response modifier (BRM) therapy or immunotherapy.

Treatment by type

Treatment for myeloproliferative disorders depends on the type of myeloproliferative disorder, whether the patient has symptoms or not, and the patient's age and overall health.

Standard treatment may be considered because of its effectiveness in patients in past studies, or participation in a clinical trial may be considered. Most patients with myeloproliferative disorders are not cured with standard therapy and some standard treatments may have more side effects than are desired. For these reasons, clinical trials are designed to find better ways to treat cancer patients and are based on the most up-to-date information. Clinical trials are ongoing in most parts of the country for the myeloproliferative disorders. To learn more about clinical trials, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237); TTY at 1-800-332-8615.

Chronic Myelogenous Leukemia

Refer to the PDQ summary on Chronic Myelogenous Leukemia Treatment for more information.

Polycythemia Vera

Treatment may be one or more of the following:

  1. Phlebotomy from time to time to lower the amount of blood in the body.
  2. Chemotherapy with or without phlebotomy.
  3. Biological therapy.
  4. A clinical trial of new treatment.

Chronic Idiopathic Myelofibrosis

Treatment may be one or more of the following:

  1. If there are no symptoms, treatment may not be needed. The doctor will follow the patient closely so treatment can be started if symptoms develop. A blood transfusion may be required if severe anemia develops.
  2. Biologic therapy.
  3. Surgery to remove the spleen (splenectomy).
  4. External radiation therapy to the spleen, chemotherapy, or biologic therapy to reduce the pain caused by the enlargement of the spleen.
  5. Chemotherapy to lower the number of platelets in the blood.
  6. Bone marrow or peripheral stem cell transplantation.
  7. A clinical trial of new treatment.

Essential Thrombocythemia

Treatment may be one or more of the following:

  1. If there are no symptoms and the patient is younger than 60 years, treatment may not be needed. The doctor will follow the patient closely so treatment can be started if symptoms develop.
  2. Chemotherapy to lower the number of platelets in the blood.
  3. Plateletpheresis to remove extra platelets from the blood.
  4. A clinical trial of biological therapy.
  5. A clinical trial of new treatment.

Chronic Neutrophilic Leukemia

Treatment for this disease remains unclear. Patients are encouraged to enroll in clinical trials evaluating new treatments.

Chronic Eosinophilic Leukemia

Treatment for this disease remains unclear. Patients are encouraged to enroll in clinical trials evaluating new treatments.

Changes to This Summary (05/20/2003)

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.

Changes were made to this summary to match those made to the health professional version.

To Learn More

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.

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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.

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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.

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About PDQ

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.

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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.


Source: National Cancer Institute
Cache Date: December 10, 2004

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