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Myeloproliferative Disorders General Information 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 four types of myeloproliferative disorders: chronic myelogenous leukemia, polycythemia vera, agnogenic myeloid metaplasia, and essential thrombocythemia. Chronic myelogenous leukemia affects the cells that are developing into white blood cells, called granulocytes. 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. Agnogenic myeloid metaplasia 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. 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 Information 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 agnogenic myeloid metaplasia, 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. 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 or P32 radiation therapy to lower the number of red blood cells. 3. Biological therapy. Agnogenic Myeloid Metaplasia 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 chemotherapy drugs. Essential Thrombocythemia Treatment may be one or more of the following: 1. Chemotherapy to lower the number of platelets in the blood. 2. Plateletpheresis to remove extra platelets from the blood. 3. A clinical trial of biological therapy. The information on this page was obtained from the National Cancer Institute. The National Cancer Institute provides accurate, up-to-date information on many types of cancer, information on clinical trials, resources for people dealing with cancer, and information for researchers and health professionals. The National Cancer Institute is in no way affiliated with the Mary Stolfa Cancer Foundation. The information on this web site is provided for general information only. It is not intended as medical advice, and should not be relied upon as a substitute for consultations with qualified health professionals who are familiar with your individual medical needs. The MSCF disclaims all obligations and liabilities for damages arising from the use or attempted use of the information, including but not limited to direct, indirect, special, and consequential damages, attorneys' and experts' fees and court costs. 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