Myeloproliferative Neoplasms

What are myeloproliferative neoplasms?

Myeloproliferative neoplasms (MPN) are a group of blood cancers in which a person’s bone marrow makes too many of a certain type of blood cell, or makes blood cells that don’t mature properly. The type of blood cell being overproduced — including platelets, white blood cells or red blood cells — determines which type of myeloproliferative neoplasms a person has.

There are six main types of myeloproliferative neoplasms

• Chronic myelogenous leukemia, in which the bone marrow makes too many white blood cells.
• Polycythemia vera, in which the bone marrow overproduces red blood cells, while sometimes also increasing the number of white blood cells and platelets. Polycythemia vera can make the blood thick, and cause swelling in the spleen. The condition can also cause clots to form in blood vessels, which can raise a person’s risk of stroke or heart attack. 
• Primary myelofibrosis (AKA chronic idiopathic myelofibrosis), in which blood stem cells don’t mature properly and the fibrous tissue inside the bone marrow becomes scar-like and thick. These improperly-matured cells, called “blasts,” can build up and inhibit your bone marrow’s ability to make new blood cells. 
• Essential thrombocythemia, in which the bone marrow makes too many platelets, which may stick together, block blood vessels or form clots. This can cause bleeding issues and increase the risk of stroke or heart attack. 
• Chronic neutrophilic leukemia, in which too many blood stem cells become neutrophils, which are infection-fighting cells that help destroy bacteria and rid the body of dead cells.
• Chronic eosinophilic leukemia, in which the bone marrow makes too many eosinophils, a type of infection-fighting cell that primarily reacts to allergens and helps fight off certain microscopic parasites.

What are the symptoms of myeloproliferative neoplasms?

The symptoms of each form of myeloproliferative neoplasms are different. Some forms of the disease present no symptoms in early stages, or symptoms that are also associated with other conditions. 

According to the National Cancer Institute:

Chronic myelogenous leukemia symptoms can include: 

• Unexplained or recurring headaches
• A feeling of fullness in the abdomen, particularly on the left side below the ribs
• Stroke
• Heart attack

Polycythemia vera symptoms can include: 

• Unexplained weakness
• Unexplained or recurring dizziness
• Unexplained weight loss
• Headaches
• Double vision or seeing dark spots or blind spots
• Full-body itching, especially after a hot or warm shower or bath
• A feeling of fullness in the abdomen, particularly on the left side below the ribs

Primary myelofibrosis symptoms can include:

• Unexplained, recurring fever
• Extreme night sweats
• Unexplained fatigue
• Anemia
• Shortness of breath
• Unexplained weight loss
• Petechiae, which are red, pinprick-sized spots of blood under the skin
• Feeling full earlier than usual while eating

Essential thrombocythemia symptoms can include:

• Unexplained headaches
• Unexplained vision or hearing problems
• Fever or redness in the hands and feet
• A burning or tingling sensation in the hands and feet

Chronic neutrophilic leukemia symptoms can include: 

• Swelling of the liver and spleen, which can cause a feeling of fullness on the left side of the abdomen
• Explained fever or sore throat
• Unexplained weight loss

Chronic eosinophilic leukemia symptoms can include: 

• Unexplained fever
• Recurring cough
• Swelling around the eyes, lips, in the throat or in the hands and feet
• Unexplained muscle soreness
• Severe fatigue
• Unexplained itching
• Diarrhea

What are the risk factors for myeloproliferative neoplasms?

The search for the root cause of myeloproliferative neoplasms is ongoing. However, there are a few factors that are thought to increase your risk of developing different types of myeloproliferative neoplasms: 

• Living with a large family as a child
• Growing up in a low-income household
• Obesity
• A history of heavy tobacco smoking
• A history of multiple CT scans 
• Being a carrier of certain genetic mutations

How can I reduce my risk of developing myeloproliferative neoplasms? 

Currently, there’s no known way to definitively prevent myeloproliferative neoplasms. However, ways to reduce your risk of developing myeloproliferative neoplasms may include:

• • Avoid being overweight by eating a healthy diet and getting regular exercise. Here at Hoag, we offer help with weight management to help reduce the risk of myeloproliferative neoplasms and other serious conditions that are linked to being overweight.

• Don’t smoke or use tobacco products

How are myeloproliferative neoplasms diagnosed?

Myeloproliferative neoplasms are most often diagnosed through blood testing after patients go to their doctor with symptoms they can’t explain.

After discussing your symptoms, your doctor will likely order what’s called a complete blood count (CBC), which counts the different types of cells in your blood. Your doctor may also request what’s known as a peripheral blood smear, which determines if your blood contains abnormally-shaped or improperly matured cells. Your blood may also be tested for certain genetic mutations that can lead to different types of myeloproliferative neoplasms. 

Depending on the results of those tests, your doctor may request what’s called a bone marrow aspiration and biopsy. In this procedure, a hollow needle is inserted through the skin and into the hip bone or breastbone so that a sample of bone marrow can be removed for examination in a lab. This sample may be subjected to what’s known as cytogenetic analysis, which inspects the chromosomes in the sample to see if they’re broken, missing or otherwise abnormal.   

Certain types of myeloproliferative neoplasms have unique methods of diagnosis. Polycythemia vera, for example, is often diagnosed through what’s called a serum erythropoietin test, which checks levels of the hormone erythropoietin in the blood. In patients with polycythemia vera, levels of this hormone would be lower than in a healthy person, because the body has an overabundance of red blood cells. 

I’ve been diagnosed with myeloproliferative neoplasms. Now what do I do?

With a constellation of possible blood cancers, each with their own symptoms and treatment options, timely, correct diagnosis is key to your effective treatment. 

No hospital in California is more committed to accuracy in diagnosis than Hoag, even with rare blood cancers like myeloproliferative neoplasms. At Hoag Family Cancer Institute, our blood cancers team includes medical oncologists who specialize in hematology, radiation oncologists, advanced endoscopists and board-certified surgeons, all working to provide you not just with treatment, but the right treatment for you. Learn more about Hoag’s specialized blood cancers team. 

Hoag’s Clinical Nurse Navigator Program

Hoag is Southern California’s leader in peace of mind for cancer patients through our Clinical Nurse Navigator program. This unique program provides cancer patients and their families with access to nurses who have specialized training in specific cancers, allowing them to serve as facilitators and guides throughout diagnosis and treatment.  

By answering questions, providing support and serving as a trusted source of science-backed information at each phase of treatment, Hoag’s Clinical Nurse Navigators are a critical resource for patients. Find more information here about Hoag’s hematological cancer clinical nurse navigator program.

What are the treatment options for myeloproliferative neoplasms?

Currently, there’s no known cure for the various forms of myeloproliferative neoplasms. However, treatments are available to lessen the impact of some of the symptoms, and new approaches are being developed every day.

At Hoag Family Cancer Institute, our multidisciplinary team offers globally-recognized options for those facing this challenging disease, with treatments tailored to meet each patient’s specific needs. At Hoag, treatments to lessen or control the symptoms of myeloproliferative neoplasms may include: 

• Phlebotomy, in which blood is drawn from the body as a way to remove excess red blood cells
• Platelet apheresis, which puts a patient’s blood through a special machine that removes excess platelets before returning the treated blood to the patient’s body
• Transfusion, in which blood is intravenously given to the patient to replace certain blood cells of which they may have too few
• Splenectomy, which is a surgery to remove the spleen if it is enlarged
• Surveillance, in which physicians closely monitor a patient’s condition to see if it advances, recedes or stays the same. This can be very helpful in planning a treatment course. 

• Radiation therapy, which uses radiation to kill cancer in the body or keep it from growing. Radiation therapy is often used in combination with chemotherapy. 
• Chemotherapy uses drug therapy to help destroy cancer cells. Chemotherapy is often combined with radiation therapy. In individuals with advanced myeloproliferative neoplasms, chemotherapy may be used alone or it may be combined with targeted drug therapy.
• Targeted therapy and precision medicine, utilizes DNA testing of patients tumors, to help select and formulate drugs that attack specific abnormalities within cancer cells. This can result in less damage to health tissues.  For example, Tyrosine kinase inhibitor (TKI) therapy blocks signals that cancerous cells need to grow. Explore precision medicine at Hoag. 

Advanced treatment for myeloproliferative neoplasms at Hoag

Myeloproliferative neoplasms are rare, and there are widely-differing opinions on effective treatments for the various types of the disease. Diagnosed with myeloproliferative neoplasms? You need the skill, technology and experience that can only be provided by a major, highly-experienced cancer center. You need Hoag. 

At Hoag, our team has seen and treated more myeloproliferative neoplasm cases than any other center in Southern California. That means we’ve got the experience and skill that can make all the difference for you, especially when facing rare and challenging cancers like myeloproliferative neoplasms. When you’re ready to fight, Hoag’s here to help.

Integrated Cancer Support Services at Hoag 

At Hoag Family Cancer Institute, we’re committed to offering the best support services, resources and educational information to our patients and their families at every stage of their cancer journey. Hoag’s Integrated Cancer Support Services are open to everyone regardless of their prognosis, cancer stage or phase of recovery. 

Resources include: 

• Classes & Workshops
• Counseling Services
• Image Appearance Enhancement Program
• Support Groups
• Hoag Rehabilitation Services
• Spiritual Care Services
• Oncology Massage
• Oncology Nutrition
• Financial Navigators for cancer-related care 

Find more information about Integrated Cancer Support Services at Hoag.

Survivorship Support at Hoag

At Hoag, we understand that a cancer diagnosis may cause you and your family to experience a variety of significant life changes. We are committed to providing you with support before, during and after treatment. Hoag’s survivorship resources help you navigate through the challenges of telling friends and family about your cancer, working while living with cancer, returning to “normal” after cancer and so much more. We strive to provide you with the resources needed to feel empowered in your everyday life.

Learn more about our Survivorship Support here.