Myeloproliferative disorder, or myeloproliferative neoplams, can be challenging to diagnose and treat, often requiring years of follow-up care and treatment. At UofL Health – Brown Cancer Center, a team of experts, which may include hematologists, pathologists, advanced practice nurses and physician's assistants, as well as a specially trained support staff, plans your treatment for myeloproliferative disease by drawing upon the very latest advancements and therapies.
It all begins with precise diagnosis by our specialized pathologists, who use advanced technology and skill to pinpoint the exact disorder. We have extensive expertise in diagnosing myeloproliferative disorder, as well as the latest technology.
Our level of experience and expertise can make a profound difference in your treatment. Our experts design a care plan specifically for you, drawing upon the most advanced options with the least impact on your body.
At Brown Cancer Center, you also benefit from an active research program. We offer a range of clinical trials of innovative treatments.
Facts
Myeloproliferative disorder has a wide range of symptoms and no known cause.
Myeloproliferative disorders can strike at any age, and they have no known cause. They have a wide range of symptoms and outlooks. Sometimes the disease progresses slowly and requires little treatment; other times it develops into acute myeloid leukemia (AML).
Myeloproliferative disorder types
There are several types of myeloproliferative disease. The main types are:
Primary Myelofibrosis affects the red blood cells and granulocytes, a type of white blood cell. The cells don't mature normally and are irregularly shaped. Primary myelofibrosis also causes thickening or scarring of the fibers inside bone marrow, which can decrease the production of red blood cells and cause anemia.
Polycythemia Vera (PV) is caused by the overproduction of red blood cells in the bone marrow, which then build up in the blood. Often, the spleen swells as extra blood cells collect there, causing pain or a full feeling on the left side. Itching all over the body is a symptom of PV.
Essential Thrombocythemia (ET) means that the number of platelets in the blood is much higher than normal, while other blood cell types are normal. The extra platelets make the blood "sticky," which slows down blood flow.
Chronic Eosinophilic Leukemia/Hypereosinophilic Syndrome (HES) is characterized by a higher than normal number of the white blood cells responsible for fighting allergic reactions and some parasitic infections (eosonophils). You may experience itching, swelling around the eyes and lips or swollen hands and/or feet. In some patients, HES may quickly progress to acute myelogenous leukemia.
Systemic Mastocytosis (SM) affects mast cells, which are found in skin, connective tissue and in the lining of the stomach and intestines. Mast cells serve as a sort of alarm system by signaling disease-fighting blood cells to target areas of the body where they're needed. They may also play a role in wound healing. SM is caused by too many mast cells accumulating in the body's tissues, which can eventually affect the spleen, bone marrow, liver or small intestine.
Risk factors
Anything that increases your chance of getting myeloproliferative disease is a risk factor. These include exposure to:
- Intense radiation, such as a nuclear bomb
- Petrochemicals, such as benzene or toluene
- Electrical wiring
- Many people with myeloproliferative disease have a mutation in the JAK2 gene. This gene mutation is acquired, which means you are not born with it.
Some cases of myeloproliferative disorder can be passed down from one generation to the next. Genetic counseling may be right for you.
Symptoms
Many times, especially in the early stages, myeloproliferative disorder does not have symptoms. When it does have signs, they vary from person to person. If you have symptoms, they may include:
- Headache
- Fatigue
- Shortness of breath
- Easy bruising or bleeding
- Petechiae (tiny red spots under the skin)
- Unexplained weight loss
- Night sweats
- Fever
These symptoms do not always mean you have myeloproliferative disorder or neoplasms. However, it is important to discuss any symptoms with your doctor, since they may signal other health problems.
Diagnosis
We have extensive expertise in diagnosing myeloproliferative disorder, as well as the latest technology. Our hematopathologists specialize in myeloproliferative disorders, giving them the knowledge to pinpoint your exact diagnosis.
Diagnosis of myeloproliferative disorder can be challenging. It often does not have symptoms in the early stages, and when it does they are often mistaken for signs of other diseases. A doctor must have a high degree of experience in MPD to make an accurate diagnosis.
If MPD is suspected, blood tests will be ordered. The blood test results can validate the suspicion, but a bone marrow biopsy may be required to definitively confirm the diagnosis.
Our experts are highly specialized in diagnosing every type of myeloproliferative disorder. We welcome the opportunity to provide second opinions for myeloproliferative disorders.
Treatment
As an active center for the care of myeloproliferative disorder, we have a level of experience and expertise that can make a profound difference in your treatment. Our experts design your personal care plan specifically for you, drawing upon the most advanced options with the least impact on your body.
Myeloproliferative disorder is often challenging to treat, and it may require years of therapy and follow-up care. Treatments for MPD generally are aimed at controlling disease symptoms, and your symptoms will help the doctor decide which treatment is best. Therapies may include one or more of the following.
Medications: Aspirin, hydroxyurea, anagrelide and interferon-alpha are the main medications for essential thrombocytemia and polycythemia vera. Thalidomide, steroids and other hormones, and cladribine and busulfan also may be used.
Blood or platelet transfusion
Growth factors, which stimulate growth of bone marrow cells, are given as injections under the skin and may benefit patients with low blood cell counts.
Radiation therapy
Surgical removal of the spleen
Phlebotomy
