Myeloproliferative Neoplasms: What Your Patients Need to Know

Myeloproliferative neoplasms (MPNs), while rare, are incredibly serious and stressful. They are a form of leukemia, but there is no known cause of an MPN beyond gene mutations; and they are not generally known to be passed down familially. It has been estimated that approximately 20,000 people are diagnosed with an MPN every year, and these cases have the potential to develop into more aggressive forms of leukemia.¹

Patients at risk of an MPN probably know little, if anything, about the types of disorders. Here are the basics you can discuss with them?

Types of Myeloproliferative Neoplasms

MPNs are a particularly rare form of blood cancer in which a person’s bone marrow creates too many red blood cells, white blood cells, or platelets. The type of MPN will be determined by which blood cell is affected or, if multiple types of cells are affected, which one is most affected.²

The types of MPNs include:

• Polycythemia vera. Patients with polycythemia vera have a notable increase of red blood cells, which can cause the blood to thicken. Polycythemia vera also puts patients at risk of bleeding and clotting problems.
• Essential thrombocythemia. Essential thrombocythemia is when the bone marrow produces an increased amount of platelets, which can cause clotting-based complications and increase patients’ risk for heart attack and stroke.
• Chronic myelogenous leukemia. Patients with chronic myelogenous leukemia have an overabundance of immature white blood cells (myeloblasts) in the bone marrow. Symptoms, which tend to present late in the disease’s progression, include bleeding more easily, fatigue, weight loss, pale skin, and night sweats.
• Primary myelofibrosis. Primary myelofibrosis is a bone marrow blood cancer that is quite rare. Abnormal blood cells and tissue build up in the bone marrow, negatively affecting its ability to produce healthy blood cells.

Common Symptoms With Myeloproliferative Neoplasms

Patients with MPNs may not experience symptoms at first, but common ones they may experience in time may include:

• Fatigue and weakness
• Headaches
• Unexplained weight loss
• Dizziness
• Itchiness

Because different MPNs affect different blood cells, patients’ symptoms can vary. Patients with essential thrombocythemia may experience vision changes and redness or warmth in the hands and feet³ while those who have polycythemia vera may experience the side effects of an enlarged spleen — which include feelings of fullness even when not eating, fatigue, anemia, and easy bleeding.⁴

How Do You Test for MPNs?

If a patient or physician suspects an MPN, a full physical and examination of their health history will be warranted. From there, blood tests will be done, and samples will be used to determine whether the amount and state of blood cells are abnormal. 

If needed, medical professionals may also perform a bone marrow aspiration and biopsy to search for abnormal cells within the blood, bone, and bone marrow. Additional blood or bone marrow testing may also be done to determine if there are any other genetic mutations that are commonly found in patients with MPNs.

How to Treat for MPNs

Treatment will depend on the type of MPN, as well as how serious the patient’s case is. If an MPN is detected but the individual is not showing any symptoms, medical professionals may choose to do watchful waiting, carefully monitoring the disease so that treatment can begin quickly — when and if symptoms start appearing.

Many patients with MPNs will require chemotherapy to stop the cancer cells from spreading, and some will need blood transfusions to replace the blood cells affected by either the disease or the chemotherapy.² Patients undergoing chemotherapy may also get stem cell transplantation, as these new, immature cells are able to mature into healthy blood cells.⁵

References

1. Myeloproliferative neoplasms (MPN). Leukemia and Lymphoma Society. https://www.lls.org/research/mpn-research-funded-by-lls. Accessed March 18, 2021.
2. Chronic myeloproliferative neoplasms treatment (PDQ®)-Patient version. National Cancer Institute. https://www.cancer.gov/types/myeloproliferative/patient/chronic-treatment-pdq. Updated October 1, 2020. Accessed March 19, 2021.
3. Essential thrombocythemia – symptoms and causes. Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/essential-thrombocythemia/symptoms-causes/syc-20361064. Accessed March 19, 2021.
4. Polycythemia vera – symptoms and causes. Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/polycythemia-vera/symptoms-causes/syc-20355850. Accessed March 19, 2021.
5. Stem cell transplantation for myeloproliferative disorders. NYU Langone Health. https://nyulangone.org/conditions/myeloproliferative-disorders/treatments/stem-cell-transplantation-for-myeloproliferative-disorders. Accessed March 19, 2021.