Glossary of Common Terms in MPNs

 

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Agrylin (anagrelide HCI): A drug therapy for essential thrombocythemia (ET) that inhibits the production of platelets.

Allogeneic: Genetically different tissues or cells that, despite being from individuals of the same species, are immunologically compatible. 

Alternative medicine: A group of diverse practices used as therapy in place of conventional Western medicine. Examples of alternative medicine include acupuncture, herbal remedies, homeopathy and meditation.  When alternative medicine is used in conjunction with traditional Western medicine, it is referred to as complementary medicine.

Anemia: Low red blood cell count in the body, which results in inadequate amounts of oxygen being delivered to body tissues.  Symptoms of anemia include fatigue, pale skin and shortness of breath. 


Blood clot (or thrombus, or thrombosis):  Blood forms a clot when it comes in contact with a “thrombogenic” substance causing the blood to convert from a liquid to a solid state (also known as coagulation). Thrombogenic substances include collagen, tissue factor and von Willebrand factor.

Bone marrow: The soft, spongy, vascular tissue found inside the bones. Bone marrow produces red blood cells through a process called hematopoiesis.

Bone marrow biopsy (or bone marrow aspiration):  Removal of the soft tissue (marrow) found inside the bone. The bone marrow biopsy is used to test for disease or disease progression. 

Bone marrow transplant (BMT):  A procedure in which healthy bone marrow stem cells are used to replace diseased or damaged bone marrow. Also called a stem cell transplant. 

Blood cancer: An uncontrolled growth of abnormal, or malignant, blood cells that affect the function and the production of blood cells in the body.

 

Chemotherapy: A category of treatment for cancer treatment that uses chemical substances or drugs to destroy cancer cells. Chemotherapy can be administered intravenously or orally. 

Coagulation:  Blood clotting.  The process by which the blood converts from a liquid to a solid state, caused by a thrombogenic substance.

Complete blood count/CBC: A comprehensive blood test used to measure the number of white blood cells and red blood cells, the amount of hemoglobin, and the level of hematocrit.  The CBC also gauges the platelet count and size of platelets. 

Corticosteroid:  A man-made drug similar to steroid hormones produced by the adrenal glands that reduce inflammation and fight illness.

Cytogenetic analysis or Karyotype:  Examining samples of blood or bone marrow to study the chromosomes in order to identify genetic disorders.

Cytokine:  A small protein released by cells that act as mediators between cells.


Deep vein thrombosis (DVT):  A blood clot that forms in a deep vein of the body, usually the thigh or leg.  If and when the clot breaks off and moves through the bloodstream, it becomes an embolism, which can get lodged in the brain, heart or lungs, causing severe damage.

 

Embolism:  A blood clot that breaks off and moves through the bloodstream, lodges in a blood vessel and blocks it.  An embolism can become lodged in the brain, heart, lungs or other area, causing severe damage.

Essential thrombocythemia:  A blood disorder, characterized by the overproduction of platelets (thrombocytes) in the bone marrow. Also referred to as primary thrombocytosis.  Essential thrombocythemia is one of the myeloproliferative neoplasms (MPNs).

 

Fibrosis:  Formation of excess connective tissue. 


Gene mutation:  A change in the DNA sequence.  Gene mutations that are often associated with MPNs include JAK2V617F mutation, MPL mutation and calreticulin (CALR) mutation. 


Hematocrit (HCT):  The proportion of red blood cells to the volume of whole blood. Abnormally high or low HCT may indicate disease.

Hematologist:  A physician specializing in blood cancers.

Hematopoietic stem cell:  Self-renewing cells that can develop into any type of specialized blood cell. 

Hemoglobin:  The molecule in the red blood cell that carries oxygen from the lungs to the body tissues.

Hemorrhage:  Excessive bleeding or the abnormal flow of blood.  Hemorrhage can be internal or external. 

Heparin:  An anticoagulant (anti-clotting) medication used to decrease blood clots, often administered as an injection.

Hepatomegaly:  Enlarged liver. 

Hydrea (hydroxyurea):  A mild chemotherapy drug that can be used to lower red blood cells, white blood cells and platelets. 

Hypertension:  High blood pressure.

 

Immunomodulators:  Medications used to help regulate or normalize the immune system.

Immunosuppressant (or immunodepressant):  An agent that can suppress the strength of the body’s immune system. Immunosuppressants are often administered post-transplant to prevent rejection of the donor organ and to treat autoimmune diseases. 

Interferon:  A protein produced by the body in response to an infection.

 

JAK inhibitor:  A medication that blocks the activity of one or more of the JAK enzymes (JAK1, JAK2, JAK3, TYK2). JAK inhibitors are used to treat certain types of cancer and inflammatory conditions.

Jakafi (ruxolitinib):  A JAK inhibitor currently approved to treat patients with intermediate or high-risk myelofibrosis (MF), including primary MF, post-polycythemia vera MF and post-essential thrombocythemia MF. Jakafi is an oral therapy.

JAK2 (JAK2v617F):  JAK2 is a gene that exists in all people and promotes the growth and division of cells. In 2005 a mutation was discovered (known as JAK2V617F) in the JAK2 gene in people with myeloproliferative neoplasms (MPNs). This mutation affects the proper signaling of the JAK2 molecule.

 

Leukapheresis:  A laboratory procedure that removes abnormal white blood cells and returns the rest of the blood cells and plasma to the body. This procedure can quickly lower blood counts, though it is only a short-term solution.

Lymphocyte:  A type of white blood cell (leukocyte) that is responsible for the immune response and aids in defending the body against disease. There are two primary types of lymphocytes: B cells and T cells.

 

Myelofibrosis (MF):  A disorder of the bone marrow that disrupts normal production of blood cells. It causes excessive scarring in the bone marrow.  Symptoms include enlarged spleen and anemia. Myelofibrosis is one of the myeloproliferative neoplasms (MPNs).

Myeloproliferative neoplasms (MPNs):  A group of diseases of the blood and bone marrow which causes overproduction of red blood cells, platelets, or certain white blood cells. There are three classic types of MPNs: myelofibrosis (MF), essential thrombocythemia (ET) and polycythemia vera (PV). 

Myelosuppressive agent:  Hinders bone marrow activity in order to decrease the production of blood cells and platelets. 

 

Neoplasm:  An abnormal, often uncontrolled, growth of tissue that results when cells divide more than they should or do not die when they should

Neutropenia:  An abnormally low count of neutrophils (white blood cells that fight infection).

Neutrophil:  A type of white blood cell produced in the bone marrow. Neutrophils fight infection and attack bacteria. 

Night sweats:  Excessive sweating at night, soaking bedclothes or linens, while sleeping.

 

Petechiae:  Broken blood vessels under the skin, which appear as flat, red, pinpoint spots.

Pheresis:  A laboratory procedure, in which the blood is filtered, separated, and a specific component is retained, while the remainder is returned to the individual. 

Philadelphia chromosome:  An abnormality of chromosome 22, which is associated with chronic myeloid leukemia (CML), a myeloproliferative neoplasm.

Phlebotomy (or venesection):  Withdrawing blood from the body, usually in large amounts, for treatment purposes. Phlebotomy is a mainstay of treatment for the polycythemia vera (PV) to lower hemoglobin and hematocrit levels. 

Plasmapheresis:  A laboratory procedure in which the plasma is removed from the blood.

Platelet:  Blood cells that aid in clotting of the blood when necessary. Platelets are also called thrombocytes.

Platelet count:  A test to measure the number of platelets found in the blood.

Plateletpheresis:  A laboratory procedure in which the platelets are removed from the blood.

Polycythemia vera (PV):  A disease of the bone marrow that leads to an overproduction of blood cells, particularly red blood cells.  White blood cells and platelets may also increase. Polycythemia vera is one of the myeloproliferative neoplasms (MPNs).

Prednisone:  An oral, synthetic steroid that acts as an immunosuppressant and can reduce inflammation.

Pruritus:  Severe itching.

Pulmonary embolism:  The obstruction of one or more of the pulmonary arteries in the lungs, caused by a blood clot that has traveled from somewhere else in the body.

 

Radiation therapy:  A type of treatment that uses beams of intense energy to kill cancer cells.

Red blood cells (RBCs):  Blood cells that carry oxygen to the rest of the body. Hemoglobin is the molecule in the red blood cell that permits the cell to transport oxygen.  Also called erythrocytes.

 

Spleen:  An organ that fights infection and keeps the body fluids in balance.  It is part of the lymphatic system and is located on the left side of the body just above the stomach and below the diaphragm.  

Splenomegaly:  Enlarged spleen.

Stem cell:  A cell that can develop into specialized cells or divide to produce more stem cells.

Stem cell transplant (SCT):  A procedure in which healthy bone marrow stem cells are use to replace diseased or damaged bone marrow. Also called a bone marrow transplant.

Stroke:  Occurs when blood flow to the brain stops or there is a rupture in a blood vessel of the brain, causing a loss of brain function.

 

Thrombocythemia:  A higher than normal number of platelets in the blood. 

Thrombocytopenia:  A lower than normal number of platelets in the blood.

Thromboembolism:  When a blood clot breaks loose and is carried through the bloodstream to plug another vessel. 

Thrombosis:  The formation of a blood clot in a blood vessel causing a total or partial obstruction of a vein or artery.

Tyrosine kinase inhibitors (TKIs):  A targeted cancer therapy that interferes with the signals that tell a cell to grow and divide. This therapy can reduce or stop cancer cells from growing, and, in some cases, it causes the cell to die.

 

White blood cells (WBCs):  Blood cells that are part of the immune system and also help fight infection. Also called leukocytes.


Page last updated on March 3, 2015
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