National Organization for Rare Disorders, Inc.
It is possible that the main title of the report Essential Thrombocythemia is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
- essential hemorrhagic thrombocythemia
- essential thrombocytosis
- idiopathic thrombocythemia
- idiopathic thrombocytosis
- primary thrombocythemia
Essential thrombocythemia, also known as ET, is a rare disease. The most important first fact about ET: on average, people with ET have a normal life expectancy.
Patients with ET have increased numbers of platelets. Platelets are the smallest of the three types of blood cells and are needed for successful blood clotting after an injury. The two other types of blood cells are red blood cells, which carry oxygen to all tissues in the body, and white blood cells, which help to fight infections. Red blood cell numbers (often measured as a percentage of whole blood, called a hematocrit) are generally normal in ET, while white blood cell numbers are normal or slightly elevated in ET. Importantly, most people with an elevated platelet count do not have ET. Common alternative causes of an elevated platelet count are iron deficiency, infection or generalized inflammation; less common causes are blood disorders such as ET or other related blood diseases (also see below).
In the 1950's, a pioneering hematologist, William Dameshek, placed ET within a family of blood diseases called myeloproliferative disorders. These were unified by their propensity to lead to abnormal increases in various blood cells, perhaps, in Dr. Dameshek's words, "due to a hitherto undiscovered stimulus". At the time, it was unclear if these "proliferations" represented a natural response to some external cause, or were the result of an internal defect.
Over time, it became obvious that the myeloproliferative disorders are caused by genetic accidents (an internal defect) in very early blood cells (stem cells), which are then passed along to all of the progeny of that cell, even as they mature into platelets, red cells, or white blood cells (see below). In acknowledgement of this new understanding, myeloproliferative disorders have been renamed myeloproliferative neoplasms (MPN). For this reason, ET is best thought of as a chronic type of leukemia - albeit one with an overall excellent prognosis and often requiring minimal or no treatment.
Leukemia & Lymphoma Society
1311 Mamaroneck Avenue
White Plains, NY 10605
American Cancer Society, Inc.
250 Williams NW St
Atlanta, GA 30303
NIH/National Heart, Lung and Blood Institute
P.O. Box 30105
Bethesda, MD 20892-0105
NIH/National Heart, Lung and Blood Institute ~ Hematology Branch
10 Center Dr, Building 10-CRC
Bethesda, MD 20892-1202
Platelet Disorder Support Association
133 Rollins Avenue, Suite 5
Rockville, MD 20852
Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
CMPD Education Foundation
P.O. Box 4758
Scottsdale, AZ 85261
Myeloproliferative Disease Support and Daily Email Digest
2011 Flagler Ave.
Key West, FL 33040
MPN Research Foundation
180 N. Michigan Avenue, Suite 1870
Chicago, IL 60601
MPN Education Foundation
P O Box 4758
Scottsdale, AZ 85261
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It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report
This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.
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Last Updated: 5/15/2014
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