Myeloproliferative neoplasms are a group of diseases in which the bone marrow makes too many red blood cells, white blood cells, or platelets. The neoplasms usually get worse slowly as the number of extra blood cells increases. Chronic myeloproliferative neoplasms sometimes become acute leukemia, in which too many abnormal white blood cells are made.

There are six types of chronic myeloproliferative neoplasms:

  • Chronic myelogenous leukemia
  • Polycythemia vera
  • Primary myelofibrosis (also called chronic idiopathic myelofibrosis)
  • Essential thrombocythemia
  • Chronic neutrophilic leukemia
  • Chronic eosinophilic leukemia

Treatment

There are several types of standard treatment. Your OHC doctor will help you determine the best care plan for you.

  • Watchful waiting: Watchful waiting is closely monitoring a patient’s condition without giving any treatment until signs or symptoms appear or change.
  • Phlebotomy: Phlebotomy is a procedure in which blood is taken from a vein. Sometimes phlebotomy is used as a treatment and blood is taken from the body to remove extra red blood cells. Phlebotomy is used in this way to treat some chronic myeloproliferative neoplasms, especially polycythemia vera.
  • Platelet apheresis: Platelet apheresis is a treatment that uses a special machine to remove platelets from the blood. Blood is taken from the patient and put through a blood cell separator where the platelets are removed. The rest of the blood is then returned to the patient’s bloodstream.
  • Transfusion therapy: Transfusion therapy (blood transfusion) is a method of giving red blood cells, white blood cells, or platelets to replace blood cells destroyed by disease or cancer treatment.
  • Chemotherapy: Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated.
  • Radiation therapy: Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy:
    • External radiation therapy uses a machine outside the body to send radiation toward the cancer.
    • Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer.
    • The way the radiation therapy is given depends on the type of cancer being treated. External radiation therapy is used to treat chronic myeloproliferative neoplasms, and is usually directed at the spleen.
  • Other drug therapy:
    • Prednisone and danazol are drugs that may be used to treat anemia in patients with primary myelofibrosis.
    • Anagrelide therapy is used to reduce the risk of blood clots in patients who have too many platelets in their blood. Low-dose aspirin may also be used to reduce the risk of blood clots.
    • Thalidomide, lenalidomide, and pomalidomide are drugs that prevent blood vessels from growing into areas of tumor cells.
  • Surgery: Splenectomy (surgery to remove the spleen) may be done if the spleen is enlarged.
  • Biologic therapy: Biologic therapy is a treatment that uses the patient’s immune system to fight cancer or other diseases. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against disease. This type of treatment is also called biotherapy or immunotherapy. Interferon alfa and pegylated interferon alpha are biologic agents commonly used to treat some chronic myeloproliferative neoplasms. Erythropoietic growth factors are also biologic agents. They are used to stimulate the bone marrow to make red blood cells.
  • Targeted therapy: Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Tyrosine kinase inhibitors are targeted therapy drugs that block signals needed for tumors to grow. Ruxolitinib is a tyrosine kinase inhibitor used to treat certain types of myelofibrosis.
  • High-dose chemotherapy with stem cell transplant: High-dose chemotherapy with stem cell transplant is a method of giving high doses of chemotherapy and replacing blood-forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of a donor. After the chemotherapy is completed, the stored stem cells are given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body’s blood cells.