Essential thrombocytosis overview
|
Essential thrombocytosis Microchapters |
|
Differentiating Essential thrombocytosis from other Diseases |
|---|
|
Diagnosis |
|
Treatment |
|
Essential thrombocytosis overview On the Web |
|
American Roentgen Ray Society Images of Essential thrombocytosis overview |
|
Risk calculators and risk factors for Essential thrombocytosis overview |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Soujanya Thummathati, MBBS [2]
Overview
Essential thrombocytosis (ET) or primary thrombocytosis is a rare hematologic disorder which arises from hematopoietic stem cells that give rise to megakaryocytes which in turn produce platelets (thrombocytes), that are normally involved in blood clotting. Essential thrombocytosis is characterized by overproduction of platelets in the absence of an underlying disease. Essential thrombocytosis was first defined by Emil Epstein and Alfred Goedel, two Austrian pathologists, in the year 1934 and was initially called hemorrhagic thrombocythemia.[1] Subsequently, essential thrombocytosis was classified as a myeloproliferative disorder along with chronic myelogenous leukemia (CML), polycythemia vera (PV), and chronic idiopathic myelofibrosis (CIMF).[2] Platelets are non-nucleated fragments of cytoplasm that aid in blood clotting by forming a platelet plug thus sealing off the site of injury in an effort to minimize or stop bleeding.[3] Normal platelet counts usually range from 150,000-450,000,000 ×10³ platelets/L of blood. The incidence of essential thrombocytosis is approximately 0.6-2.5 cases per 100,000 individuals worldwide annually.[4] The prevalence of essential thrombocytosis is about 30 for every 100,000 people worldwide.[5] Females are more commonly affected with essential thrombocytosis than males.[6] The female to male ratio is approximately 2 to 1.[4] Symptoms[7] of essential thrombocytosis include vision disturbances, transient loss of consciousness, chest pain, intense burning pain in hands or feet (erythromelalgia), numbness and tingling of hands and feet, persistent and painful erection of the penis (priapism). Neurologic symptoms like headache may occur but the pathophysiology is not completely understood.[7] The majority of cases of essential thrombocytosis only require supportive care. Pharmacologic medical therapies for essential thrombocytosis include aspirin therapy for patients at low risk and platelet lowering drugs (Hydroxyurea, interferon-α and anagrelide) for patients at high risk for thrombosis.
References
- ↑ Steven Sanchez & April Ewton (2006). "Essential thrombocythemia: a review of diagnostic and pathologic features". Archives of pathology & laboratory medicine. 130 (8): 1144–1150. PMID 16879015. Unknown parameter
|month=ignored (help) - ↑ Levine, R. L.; Gilliland, D. G. (2008). "Myeloproliferative disorders". Blood. 112 (6): 2190–2198. doi:10.1182/blood-2008-03-077966. ISSN 0006-4971.
- ↑ Platelet. Wikipedia. https://en.wikipedia.org/wiki/Platelet Accessed on November 11, 2015.
- ↑ 4.0 4.1 Fabris F, Randi ML (2009). "Essential thrombocythemia: past and present". Intern Emerg Med. 4 (5): 381–8. doi:10.1007/s11739-009-0284-x. PMID 19636672.
- ↑ Essential Thrombocythemia. Genetics Home Reference. http://ghr.nlm.nih.gov/condition/essential-thrombocythemia. Accessed on October 29, 2015
- ↑ Essential Thrombocythemia (ET). MPN Research foundation. http://www.mpnresearchfoundation.org/Essential-Thrombocythemia Accessed on November 15, 2015.
- ↑ 7.0 7.1 Brière JB (2007). "Essential thrombocythemia". Orphanet J Rare Dis. 2: 3. doi:10.1186/1750-1172-2-3. PMC 1781427. PMID 17210076.