About Myeloproliferative Neoplasms

Overview

Myeloproliferative neoplasms (MPNs) are a group of slow-growing blood cancers in which the bone marrow makes too many abnormal red blood cells, white blood cells, or platelets. These abnormal blood cells then accumulate in the bloodstream.

MPNs can affect different types of blood cells, but usually one type is affected more than the others. The abnormal cells may not function the same way healthy cells do. The role of healthy blood cells that may be affected by MPNs are as follows:

  • White blood cells: Help fight infection
  • Red blood cells: Carry oxygen through the body
  • Platelets: Help prevent excessive bleeding

There are six types of MPNs:

  • Chronic myelogenous leukemia (CML)
  • Polycythemia vera
  • Primary myelofibrosis
  • Essential thrombocythemia
  • Chronic neutrophilic leukemia
  • Chronic eosinophilic leukemia

MPNs are considered clonal hematopoietic disorders, meaning that they arise from a single abnormal stem cell in the bone marrow. The classification and diagnostic criteria for MPNs have evolved over time as our understanding of these conditions has improved.

Causes and Risk Factors

Myeloproliferative Neoplasms (MPNs) are a group of blood disorders that happen when genetic mutations (changes in DNA) cause blood stem cells to grow out of control. These cells mostly turn into too many white blood cells, red blood cells, or platelets.

The main reason this happens is due to the JAK/STAT pathway (a system in the body that controls cell growth) becoming too active.

Non-modifiable risk factors you cannot change:

  • Genetic mutations – The JAK2 mutation is found in most people with MPNs.
  • Age – Older adults have a higher risk, especially for blood clots.

Modifiable risk factors you can change:

  • Radiation exposure – This includes past radiation treatments for other medical conditions.
  • Other health conditions – Long-term inflammation (ongoing swelling in the body) and chronic kidney disease (permanent kidney damage) are linked to blood clots and survival rates in MPN patients.

Even if you have one or more of these risk factors, it doesn’t mean you will develop MPNs. If you're worried about your risk or have symptoms, talk to a doctor for proper testing and treatment options.

Symptoms

MPNs are usually slow growing. People may have MPNs for years without symptoms.

One common symptom across most different types of MPNs is an enlarged spleen. The symptom of this may be a feeling of fullness or pressure below the ribs on the left side

Other symptoms depend on the specific type of MPN:

  • Chronic eosinophilic leukemia: Rash, fever, and fatigue
  • Chronic myelogenous leukemia: Night sweats, fever, fatigue, easy bruising, bone pain, loss of appetite
  • Chronic neutrophilic leukemia: Night sweats, fever, fatigue, easy bruising, bone pain, loss of appetite
  • Essential thrombocythemia: Easy bruising, bleeding from nose, mouth, gums, stomach, or intestine; or blood in urine
  • Polycythemia vera: Headaches, dizziness, blurred vision
  • Primary myelofibrosis: Anemia symptoms (fatigue, shortness of breath), pale skin, fever, itchy skin, night sweats, bone pain, weight loss

It's important to note that these symptoms can vary from person to person and not all individuals will experience all of them.

If you are experiencing any concerning symptoms, it is advisable to consult with your healthcare professional for an accurate diagnosis and appropriate management.

Diagnosis

To diagnose MPN, the following examinations, tests, and procedures are commonly performed:

  • Physical exam and health history: The healthcare provider examines the body for general signs of health and checks for enlarged spleen and liver. They also gather information about the patient's health habits and past illnesses.
  • Complete blood count (CBC) with differential: A blood sample is drawn to check the number of red blood cells, platelets, and different types of white blood cells. The amount of hemoglobin in red blood cells is also measured.
  • Peripheral blood smear: A sample of blood is examined under a microscope to check for blast cells, white blood cell count, platelet count, and any changes in the shape of blood cells.
  • Bone marrow aspiration and biopsy: A small piece of bone and bone marrow is removed using a needle inserted into the hipbone or breastbone. A pathologist analyzes these samples under a microscope to look for abnormal cells.
  • Blood chemistry studies: A blood sample is checked to measure the levels of certain substances released by organs and tissues in the body. Abnormal levels can indicate disease.
  • Cytogenetic analysis: This laboratory test counts and checks the chromosomes in bone marrow or blood cells for any changes. Changes in certain chromosomes may indicate cancer.

It's important to consult with your healthcare professional about which specific examinations, tests, and procedures are necessary for your individual situation.

Treatment Options

The goals of treatment for MPNs are to manage symptoms, reduce the risk of complications such as thrombosis, and improve quality of life. The specific treatment will depend on the type of MPN and symptoms being experienced.

Here are the different types of treatments and how they work towards achieving these goals:

Medications:

  • Drug therapy: Medications may be prescribed to control blood cell production and reduce symptoms. These medications can help normalize blood counts and decrease the risk of blood clots.
  • Targeted therapy: This type of treatment focuses on specific molecular targets in MPNs to inhibit abnormal cell growth and proliferation.
  • Immunotherapy: This approach uses the body's immune system to target and destroy cancer cells.

Therapies:

  • Phlebotomy: This procedure involves removing excess red blood cells to reduce the risk of complications.
  • Platelet apheresis: It is a procedure that removes excess platelets from the blood to manage high platelet counts.
  • Transfusion therapy: Blood transfusions may be given to improve anemia or other blood-related issues.

Therapeutic Procedures:

  • Radiation therapy: It may be used in certain cases to target and destroy abnormal cells in the bone marrow.
  • High-dose chemotherapy with stem cell transplant: This procedure involves high-dose chemotherapy followed by a stem cell transplant to replace diseased bone marrow with healthy stem cells.

Health Behavior Changes:

  • Maintaining a healthy lifestyle, including regular exercise, quit smoking, a balanced diet, and stress management, can help manage symptoms and improve overall well-being.

Clinical trials:

  • Clinical trials investigate novel treatments that aim to improve current therapies or explore new options for MPN management. Consult with your healthcare professional about your specific situation to see if a clinical trial is a potential option for you.

Remember, treatment decisions should be made in consultation with your healthcare professional, as they can provide personalized recommendations based on your individual situation.

Medication dosing may be affected by many factors. Check with your health care professional about dosing for your individual situation. Side effects can occur. Check with your health care professional or read the information provided with your medication for additional side effect information.

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