Myeloproliferative Neoplasms
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What Are Myeloproliferative Neoplasms?
Our blood cells—which are responsible for blood clotting, transportation of oxygen, and immune defense against microorganisms that cause disease—are made in the marrow of our bones. Bone marrow is a spongy substance and houses the progenitor cells that give rise to our many different kinds of blood cells. In rare cases, these blood cells proliferate abnormally in the bone marrow. These cases are known as myeloproliferative neoplasms (also known as “myeloproliferative disorders”). Myeloproliferative neoplasms are classified by the specific type of cell that multiplies:
In polycythemia vera (alternatively known as “polycythemia rubra vera” and abbreviated as either “PV” or “PRV”), the bone marrow produces an abundance of red blood cells, also known as “erythrocytes.”
Essential thrombocytosis (alternatively known as “essential thrombocythemia” and abbreviated as “ET”) is a disorder characterized by the production of too many platelets, which are cell fragments involved in blood clotting.
In primary myelofibrosis (alternatively known as “idiopathic myelofibrosis,” as “myelosclerosis,” or simply as “myelofibrosis”) the normal production of blood cells is disrupted by the formation of scar tissue in the bone marrow.
Chronic myeloid leukemia (alternatively known as “chronic myelogenous leukemia” and abbreviated as “CML”) is a cancer of the bone marrow that produces abnormal white blood cells.
Chronic eosinophilic leukemia (abbreviated as “CEL”) is rare and occurs when a surplus of eosinophils is produced. Eosinophils are specialized white blood cells responsible for responding to allergic reactions and parasitic threats to the body.
Chronic neutrophilic leukemia (abbreviated as “CNL”) occurs when a surplus of neutrophils are produced. Eosinophils are specialized white blood cells responsible for responding to allergic reactions and parasitic threats to the body.
Systemic mastocytosis (alternatively known as systemic mast cell disease and abbreviated as either “SM” or “SMCD”) is a disorder characterized by an abundance of mast cells. These cells call to action the other cells of the immune system to respond to threats to the body.
Signs and Symptoms of Myeloproliferative Neoplasms
The following may be indicative of each of the following myeloproliferative neoplasms but may also be indicative of other illnesses:
Polycythemia Vera
- Abdominal bulging, discomfort, and/or pain
- Temporary blurring or loss of vision
- Blind spots
- Blood clots
- Bone pain
- Bruising
- Excessive sweating during the day or at night
- Feeling of fullness
- Gout
- Headaches and/or dizziness
- Heavy bleeding from minor injuries
- Itchiness, particularly after exposure to warm water
- Persistent fatigue
- Redness of skin
- Weakness
Essential Thrombocythemia
- Chest pain
- Bleeding
- Blood in stool
- Bruising
- Headaches and/or dizziness
- Fainting
- Feeling of fullness
- Temporary blurring or loss of vision
- Transient ischemic attack and/or stroke event(s)
- Pain, redness, swelling, tingling, and/or warmth of hands and feet
Primary Myelofibrosis
- Abdominal bulging, discomfort, and/or pain
- Bleeding
- Bone pain
- Bruising
- Shortness of breath
- Fatigue
- Feeling of fullness
- Excessive sweating during the day or at night
- Persistent or recurrent fever
- Weakness
Chronic Myeloid Leukemia
- Abdominal bulging, discomfort, and/or pain
- Fatigue
- Feeling of fullness
- Bleeding
- Bone pain
- Bruising
- Excessive sweating during the day or at night
- Persistent or recurrent fever
- Shortness of breath
- Unintentional weight loss
- Weakness
Chronic Eosinophilic Leukemia
- Diarrhea
- Fatigue
- Feeling of fullness
- Itching
- Persistent cough
- Persistent or recurrent fever/li>
- Muscle pain
- Shortness of breath
- Swollen lymph nodes
Chronic Neutrophilic Leukemia
- Abdominal bulging, discomfort, and/or pain
- Bruising
- Fatigue
- Feeling of fullness
- General discomfort
- Persistent or recurrent fever
- Throat soreness
Systemic Mastocytosis
- Allergic reactions
- Bleeding
- Cramping
- Diarrhea
- Fainting
- Headaches and/or dizziness
- Hives
- Muscle Pain
- Nausea and/or vomiting
- Shortness of breath
- Swelling
It is important you tell your doctor if you have any of these signs and symptoms, so he or she may determine their cause and plan treatment, if necessary.
How Are Myeloproliferative Neoplasms Detected?
Our specialists collect information regarding medical history, surgical history, social history, and family history; conduct laboratory testing, and review radiological studies to approach patient care in the most comprehensive and personalized manner.
The blood test normally used to assess for blood disorders is known as the “Complete Blood Count.” This test will allow your doctor to determine if you have an abnormal amount of any one or combination of blood cell types. To investigate a suspicious finding further, your doctor may choose to order a flow cytometry or bone marrow biopsy. A flow cytometry is a cell counting technique used to gather more information about cells in the blood. A bone marrow biopsy is a tissue sampling technique used to determine the composition of the bone marrow and whether or not it is involved by an abnormality, such as a myeloproliferative neoplasm.
What Causes Myeloproliferative Neoplasms?
Myeloproliferative neoplasms occur because the bone marrow produces an abnormal abundance of one or more cell types. Although the increased likelihood of developing a myeloproliferative neoplasm is associated with the following factors, in most cases, physicians and scientists are still trying to determine what causes myeloproliferative neoplasms to develop:
- Exposure to ionizing (high-energy) radiation
- Exposure to certain chemicals, such as benzene and toluene
How Are Myeloproliferative Neoplasms Treated?
Treatment of myeloproliferative neoplasms, depending on the type, may include chemotherapy, radiation therapy, surgery, and/or other therapeutic approaches. These treatments may be used individually or in combination based on your doctor’s recommendations. It’s important to discuss all of your treatment options with your doctor to help make the decision that best fits your needs. Some important factors to consider when deciding on a treatment plan include
- Your age, health, and lifestyle.
- Your specific type of myeloproliferative neoplasm.
- Any other serious health conditions you have.
- Your feelings about the need to treat the myeloproliferative neoplasm right away.
- Your doctor’s opinion about if you need to treat the myeloproliferative neoplasm right away.
- The likelihood that treatment will help fight your myeloproliferative neoplasm.
- Possible side effects from each treatment method.
You may feel the need to make a quick decision, but it is very important to ask questions if there is anything you’re not sure about. It is very important for you and your doctor to communicate and work together to weigh the benefits of each treatment option against the possible side effects and to ultimately determine what treatment option is best for you.