Myeloproliferative Disorders

Myeloproliferative Disorders

Signs and Symptoms
Diagnosis
Treatment

Treatment

Although myeloproliferative disorders (MPD) usually cannot be cured, there are treatments for all patients with the condition. Treatment of MPD depends on the subtype and the presence of symptoms. In general, treatment is aimed at correcting the abnormal blood counts. Some people don't experience any symptoms and therefore treatment may not be required for them.

Low dose chemotherapy pills (like hydrea) can be given to patients with ET and PRV to lower their red blood cell and platelet counts. Phlebotomy (the removal of blood intravenously) is also an effective strategy for lowering red blood cell counts and controlling symptoms. The treatment of choice for chronic myelogenous leukemia (CML) is the new anti-leukemia medication named Gleevec. Please see the CML section for full details. Testoterone therapy can sometimes improve the anemia in myelofibrosis (MF). Other potential therapies for MF include chemotherapy, radiation therapy, thalidomide and alpha interferon. In addition, red blood cell transfusions frequently are required for patients with MF.

Chemotherapy

Chemotherapy uses drugs to kill extra blood cells in the body. Chemotherapy may be taken orally in a pill format or may be administered intravenously (through an IV). Chemotherapy is called a systemic treatment because the drug enters the bloodstream, travels through the body and can kill cells throughout the body.

Radiation Therapy

Radiation therapy uses high-energy X-rays to kill cells and is sometimes used to relieve symptoms of MPD. External beam radiation therapy, which uses a machine outside the body to administer the radiation, is usually the type of radiation therapy used to treat MPD. In addition, a radioactive drug called P32 can be given intravenously to lower the number of red blood cells made by the bone marrow.

Surgery

Surgery to remove the spleen, called a splenectomy may be performed if a patient's spleen is swollen.

Biological Therapy

Biological therapy is being tested as a possible treatment of MPD. This type of therapy uses materials made either by the body or in a laboratory to boost, direct or restore the body's immune system. Biological therapy sometimes is called biological response modifier (BRM) therapy or immunotherapy.

Hormones

Hormones may be used in certain instances to treat side effects of MPD. In patients with agnogenic myeloid metaplasia, hormones called glucocorticoids may be given to increase the life span of red blood cells. In addition, hormones called androgens sometimes are used to stimulate the bone marrow to produce more blood cells.

A person's recovery or response to therapy depends on their type of MPD, age and general health. Most people with ET and PRV live more than 10 to 15 years with few complications. People with MF live approximately five years and in some cases, the disease may develop into acute leukemia.

Treatment Evaluation

Patients with MPD first undergo an extensive evaluation including:

• A general history and physical examination
• Multiple blood tests
• X-rays
• Possibly a bone marrow aspiration and biopsyprocedure

Patients with advanced MPD may qualify for a number of experimental therapies including stem cell transplantation. The only curative therapy for MPD (excluding chronic myelogenous leukemia) is allogeneic stem cell transplantation, which is the transfer of stem cells from one person to another.

There are several experimental studies open at UCSF Medical Center utilizing stem cell transplantation in patients with MPD. Whether stem cell transplantation is an appropriate therapy for an individual patient depends on a number of factors including age, MPD subtype, current symptoms or the extent of disease and the availability of a donor. People are encouraged to consult with a stem cell transplantation doctor to learn more about MPD, possible treatments and stem cell transplantation.

Reviewed by health care specialists at UCSF Medical Center.
Last updated May 8, 2007