Myeloproliferative Disorders

Also Listed As: Bone Marrow Disorders; Chronic Myelogenous Leukemia; Myelofibrosis; Polycythemia Vera; Thrombocytosis

Myeloproliferative disorders are a group of conditions that cause an overproduction of blood cells – platelets, white blood cells, and red blood cells – in the bone marrow. Though myeloproliferative disorders are serious, and may pose particular health risks, individuals with these conditions often live for many years after diagnosis. Myeloproliferative disorders include:

Signs and Symptoms

Many individuals with myeloproliferative disorders have no symptoms at all when their physicians first make the diagnosis. A sign that is common to all myeloproliferative disorders (with the exception of essential thrombocytosis) is an enlarged spleen, which can lead to abdominal pain and a feeling of fullness.

Some signs and symptoms specific to the different types of myeloproliferative disorders include:

Polycythemia vera Essential thrombocytosis Primary myelofibrosis Chronic myelogenous leukemia (CML)

Causes

All myeloproliferative disorders arise from an overproduction of one or more types of cells. The reason for this abnormal increase in cells is largely unknown, but there are some theories as to why they occur. These include:

Risk Factors

The following risk factors may increase an individual's risk for developing a myeloproliferative disorder:

Polycythemia vera Essential thrombocytosis Primary myelofibrosis Chronic myelogenous leukemia (CML)

Diagnosis

A sign that is common to all myeloproliferative disorders (with the exception of essential thrombocytosis) is an enlarged spleen, which can be detected during a routine physical examination. In addition to performing a physical exam, the physician may also conduct the following procedures to diagnose a myeloproliferative disorder:

Treatment Approach

Unfortunately, there are no known cures for most myeloproliferative disorders. There are, however, a number of treatments that help improve symptoms and prevent complications associated with the conditions.

The approach to treatment for each type of myeloproliferative disorder is slightly different:

Medications

An individual's diagnosis and symptoms will determine the type of medication that a physician prescribes. Some possible medications include:

Polycythemia vera Essential Thrombocytosis Primary myelofibrosis Chronic myelogenous leukemia (CML)

Surgery and Other Procedures

In the case of primary myelofibrosis, CML, and late stage polycythemia vera, blood formation occurs in sites other than the bone marrow, such as the liver and spleen, causing enlargement of these organs. When enlargement of the spleen becomes painful, a surgeon may perform a splenectomy to remove this organ.

In very serious cases of primary myelofibrosis, surgeons may replace the abnormal stem cells (cells that manufacture blood cells) in the bone marrow with healthy stem cells. This type of procedure, called a stem cell transplant, carries a high degree of risk. Another procedure, called a bone marrow transplant, is ideal for most individuals with CML. After either type of transplant, the healthy bone marrow cells circulate and begin to grow and produce healthy blood cells.

Phlebotomy may prevent the accumulation of blood and decrease the risk of stroke in individuals with polycythemia vera.

Nutrition and Dietary Supplements

Preliminary studies suggest that nutritional supplements may reduce the symptoms of some myeloproliferative disorders. These nutritional supplements include:

Vitamin A
According to the results of a seven-year study of individuals with CML, chemotherapy combined with vitamin A may be significantly more effective than chemotherapy alone in improving an individual's chance of survival. Laboratory studies also indicate that vitamin A may inhibit tumor growth in cancer cells.

Vitamin K
Laboratory studies suggest that synthetic vitamin K inhibits the growth of CML cells and enhances the effect of the chemotherapy drug, doxorubicin. Some researchers speculate that taking vitamin K while undergoing chemotherapy with doxorubicin may increase an individual's chance of survival with minimal toxic side effects. More research is needed, however, to confirm this theory.

Herbs

Although herbs have not been scientifically investigated specifically for the treatment of myeloproliferative disorders, a trained specialist may recommend the following to improve symptoms associated with the conditions:

Frankincense (Boswellia carteri)
Laboratory studies indicate that this traditional Chinese herb, normally used for improving blood circulation and relieving pain, may inhibit the growth of cancer cells. These effects, however, have been observed only in the laboratory, and may not translate to human patients.

Other Considerations

Pregnancy
The drug hydroxyurea, when used to treat myeloproliferative disorders, may pose a risk to a developing fetus and should be avoided by pregnant women.

Prognosis and Complications

Myeloproliferative disorders are slow acting, and don't always cause life-threatening symptoms. The complications of these illnesses, however may be serious. Some complications include: The survival rate for myeloproliferative disorders varies, depending on both the type of disorder and the kind of symptoms experienced by each individual. Very serious cases, such as primary myelofibrosis, may be fatal within 3 to 6 years. Individuals with CML have a median survival rate of 4 to 5 years after diagnosis. If CML transforms into acute leukemia, however, the median survival rate is only three months. Those with other types of myeloproliferative disorders can live much longer, especially if they are diagnosed early. Individuals with primary thrombocythemia have a near normal life expectancy with only a low risk of developing cancer. Polycythemia vera has a survival rate of between 10 and 20 years, with the longest survival occurring in the younger age groups.

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