Myeloproliferative disorders (MPDs) are medical conditions where bone marrow cells are produced abnormally, affecting the production of white blood cells, platelets, and red blood cells. The cause is unknown, but environmental factors and chromosomal abnormalities may play a role. Symptoms vary depending on the type of MPD, and complications can include enlarged spleen and liver, severe anemia, and bleeding tendencies. Treatment options depend on the type of MPD and may include splenectomy, phlebotomy, and stem cell transplantation.
A myeloproliferative disorder (MPD) is the collective term used for a group of medical conditions characterized by the abnormal production of bone marrow cells. Bone marrow, found within the soft tissues of larger bones, is primarily responsible for the production of white blood cells (WBCs), platelets, and red blood cells (RBCs). Red blood cells are cells that deliver oxygen to other tissues within the body. White blood cells are infection-fighting cells, and platelets help in blood clotting.
The cause of the overproduction of these blood cells is unknown, but environmental factors such as prolonged exposure to radiation or chemicals and chromosomal abnormality are said to play a role in the development of myeloproliferative disease. This disorder commonly affects men more than women between the ages of 40 and 60. Its occurrence is not common in individuals under the age of 20.
There are several types of myeloproliferative disease. These include essential thrombocythemia, myelofibrosis, polycythemia vera and chronic myeloid leukemia (CML). In essential thrombocythemia, there is an increase in platelet production. Myelofibrosis affects the production and maturity of red blood cells and white blood cells due to thickening of the fibers in the bone marrow. Polycythemia vera is the overproduction of red blood cells, and CML is characterized by increased production of white blood cells.
Symptoms generally depend on the patient’s type of myeloproliferative disease. For example, patients with polycythemia vera usually develop purple patches on the skin accompanied by itching. There are, however, several symptoms commonly presented by patients with myeloproliferative disorders. These include getting tired easily, abdominal discomfort, pale skin, bruising, and weight loss, among many others.
Complications associated with myeloproliferative disease are splenomegaly or enlarged spleen, hepatomegaly or enlarged liver, and severe anemia, a condition with very low red blood cells in the circulation. Some patients may also develop kidney failure and bleeding tendencies. Other cases can develop into acute myeloid leukemia, a malignant disease of the bone marrow that often causes very young blood cells to enter the circulation.
Patients with myeloproliferative disorders are usually managed by an oncologist and a hematologist. An oncologist is a cancer specialist and a hematologist is a blood specialist. They usually use diagnostic tests such as blood tests and bone marrow tests to confirm their diagnosis.
Treatment for myeloproliferative disorder often depends on the type. Splenectomy, or removal of the spleen, is occasionally indicated in patients with chronic idiopathic myelofibrosis. Phlebotomy, the process of drawing blood, is often done in patients with polycythemia vera. Stem cell transplantation is also an option for patients with chronic myeloid leukemia and myelofibrosis. Patients with myeloproliferative disorder are also advised to maintain hydration, avoid alcohol and smoking, and include antioxidant-rich foods in their diet.
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