Treatment Options for Myeloproliferative Neoplasms
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      / Updated January 27th, 2023

      Treatment Options for Myeloproliferative Neoplasms

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      Myeloproliferative Neoplasms Treatment

      • Until now, myeloproliferative neoplasms are considered incurable, but there are some treatment options to help improve patients’ quality of life and manage the symptoms.
      • Myeloproliferative neoplasms treatment options will depend on the type of MPN but they're generally aimed at managing symptoms of the disease. Therapeutic options include the following: Phlebotomy, blood transfusions, chemotherapy, radiotherapy, immunotherapy, targeted therapy, stem cell transplant, medications, and surgery.
      • Early diagnosis plays an important role in improving treatment response for MPN patients.
      Myeloproliferative neoplasms are a group of diseases that involve the blood and bone marrow. They’re known as MPNs because they usually cause an overproduction of one or more types of blood cells (red blood cells, white blood cells, and platelets). Although they can affect anyone at any age they mostly occur in late middle or early old age. 

      "Myeloproliferative Neoplasms (MPNs) is kind of the overarching title of these diseases and the therapy really depends on what the condition specifically is," Dr. Adrienne Philips, a Hematologist/Medical Oncologist, at Weill-Cornell Medicine, explained.

      Read More
      In this article, we're going to lay out myeloproliferative neoplasms (MPNs) treatment guidelines and therapeutic options that are currently available for managing this condition.

      Until now, myeloproliferative neoplasms are considered incurable, but several institutional bodies and research efforts are being put into changing this reality. As evident by the MPN Research Foundation saying "Helping people with an MPN live a better quality of life as we work toward answers to the prevention, progression and a cure for polycythemia vera (PV), essential thrombocythemia (ET) and myelofibrosis (MF) blood cancers collectively known as myeloproliferative neoplasms."

      What You Need to Know About Myeloproliferative Neoplasms (MPNs)

      Myeloproliferative neoplasms (MPNs) are rare, and they affect the bone marrow causing rapid growth and proliferation of blood cells. Depending on which type of blood cells is altered, they're classified into the following 6 types:

      The most common of these are polycythemia vera, primary myelofibrosis, and essential thrombocythemia. Your doctor will perform some diagnostic tests to find out which type you have so that he can choose the best treatment plan for you. These tests can include the following:

      • Medical history and physical examination
      • Complete blood count and differential 
      • Biopsy
      • Cytogenetic testing
      • Molecular testing

      What are the early symptoms of myeloproliferative neoplasms disorders?

      Symptoms of myeloproliferative neoplasms (MPNs) are different in each patient. However, they generally include the following:

      • Pale skin
      • Loss of appetite
      • Shortness of breath (especially during the time of activity)
      • Fatigue and tiredness
      • Muscle weakness
      • Prolonged bleeding
      • Frequent infections
      • Purpura a condition characterized by the formation of black and blue spots on the skin

      Left untreated, these conditions may cause acute bleeding, clotting, anemia, and other unwanted complications.

      Myeloproliferative Neoplasms Treatment Guidelines

      Myeloproliferative neoplasms treatment guidelines are aimed at managing symptoms of the disease. Therapeutic options include the following:

      • Watchful waiting this is usually prescribed when the patient doesn't exhibit any symptoms. So, they don't receive any treatment but are closely monitored for any changes.
      • Phlebotomy The process of removing blood from the body.
      • Platelet apheresis The process of removing platelets from the blood.
      • Blood transfusions The process of replacing depleted blood.
      • Surgery an operation like 'splenectomy: the removal of the spleen' can be done to alleviate symptoms of MPN.
      • Chemotherapy Chemical drugs that are given to stop the growth of abnormal MPN cells. However, they can also affect surrounding healthy cells.
      • Radiation therapy Subjecting high-energy radiation onto MPN or the spleen in some cases.
      • Immunotherapy Using certain medications to boost the body's ability to fight off MPN (ex. Interferon alpha).
      • Targeted therapy a type of medication that functions in inhibiting the growth of MPN cells. They can recognize specific features on these cells and stop their growth (they're more specific than chemotherapeutic drugs).
      • Stem cell transplant a type of procedure that introduces stem cells (immature cells that can form blood cells like in the bone marrow) into the body of an MPN patient who has previously taken chemotherapy. 
      • Other medications are drugs that inhibit specific gene or protein mutations causing the MPN in the first place (ex. JAK inhibitors).

      Polycythemia Vera Treatment

      Polycythemia vera is a condition that is characterized by increased red blood cell counts. Thus, the first treatment option to control the disease and manage the symptoms is to perform phlebotomy regularly. However, phlebotomy may also be associated with side effects including:

      • Anemia
      • Allergies
      • Thrombosis when a blood clot gets lodged in a vein or artery
      • Air embolism when an air bubble enters the arteries or veins
      • Hematoma bad bruise
      • Hyperventilation rapid breathing

      If the patient is deemed high-risk with a history of bleeding or clotting, other treatment options are considered. These include the following:

      • Cytoreductive medication chemotherapeutic drugs like hydroxyurea (Hydrea) which limit the ability of bone marrow to produce blood cells and stop its excess production.
      • Interferon to boost the immune system and reduce the production of red blood cells. Even though itching is a side effect of this medication, doctors usually prescribe an antihistamine as well to treat it.
      •  Ruxolitinib (Jakafi) is a drug that targets and binds to mutations in JAK2.

      Essential Thrombocythemia Treatment

      Essential thrombocythemia is a condition that's characterized by the production of too many platelets.  Dr. Ghaith Abu-Zeinah, an instructor in Medicine at Weill Cornell Medical College and an Assistant Attending Physician at the New York-Presbyterian Hospital, explains, "on the spectrum of the MPNs [ET] is probably the least aggressive of all subtypes". The goal of treatment is to bring down platelets level to the normal range in the body. Below is a list of the possible therapeutic options:

      • Chemotherapy these include medications that reduce platelet and alter bone marrow's ability to make and produce blood cells (ex. hydroxyurea 'Hydrea', or anagrelide 'Agrylin'.

      Side effects of these medications differ from patient to patient, but they generally include the following:

      • Fluid retention
      • Headaches
      • Fatigue
      • Dizziness 
      • Stomach upset
      • Nausea
      • Diarrhea
      • Heart problems

      Primary Myelofibrosis Treatment

      Myelofibrosis is a condition that's characterized by the formation of too many fibroblasts (the cell which makes fibrous tissue) in the bone marrow resulting in easy-destructible, fewer blood-forming cells, and anemia, low platelet count, and high susceptibility to developing infections. 

      Treatment for primary myelofibrosis largely depends on the extent of the disease, this can be decided by your doctor. Below is a list of the possible therapeutic options:

      • Watchful waiting this is suitable for patients with myelofibrosis who don't exhibit any symptoms. However, they're followed up and monitored closely.
      • Ruxolitinib (Jafaki) by targeting JAK2 mutation, this drug decreases the size of the spleen and alleviates most symptoms of myelofibrosis.
      • Radiation therapy and surgery for patients who suffer from abnormally large spleens.
      • Blood transfusions and hormone therapy are used to treat anemia associated with myelofibrosis (ex. Erythropoietin hormone).
      • Interferon and hydroxyurea are given to reduce the production of blood cells.
      • Stem cell transplantation hematopoietic stem cell transplantation or bone marrow transplantation can be potentially curative. This process involves replacing bone marrow abnormal cells with healthy cells. These healthy cells can come from the same patient (autologous transplant) or a donor (allogeneic transplant). 

      On the other hand, stem cell transplantation is associated with many side effects, these include the following:

      • Nausea
      • Vomiting
      • Mouth sores
      • Fatigue and tiredness
      • Diarrhea
      • Low levels of platelets
      • Low levels of red blood cells
      • Infections
      • Transplantation rejection
      • Bone damage
      • Lung problems
      • Liver problems

      Additionally, stem cell transplantation isn't suitable for everyone. Thus, you must consult your doctor first to check and see if you're the right candidate for this procedure.

      The Bottom Line

      Treatment of MPNs is typically a lifelong process. The types of treatment can vary depending on the type of MPN, but most treatments will include some combination of chemotherapy and medications to suppress the immune system or help manage symptoms.

      Early diagnosis of MPN can go a long way in your recovery and treatment outcomes. Thus, you should regularly follow up with your doctor.

      Questions to Ask your Doctor

      • What type of myeloproliferative neoplasm do I have? 
      • What treatment options are available for me?
      • Are there treatments to help relieve my symptoms?
      • How do I know if my treatment is working?
      • What are the risks and benefits associated with the recommended treatment option?
      • Am I eligible to receive bone marrow transplantation?

      Learn more about SurvivorNet's rigorous medical review process.

      Myeloproliferative Neoplasms (MPNs)

      There are several types of blood cancers and the treatment approach will depend on the specifics of an individual's disease, as well as that classification. Understanding your diagnosis is important.

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