Essential thrombocytosis overview: Difference between revisions
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==Overview== | ==Overview== | ||
Essential ([[ET]]) or primary thrombocytosis is a rare hematologic disorder characterized by overproduction of platelets in the absence of an underlying disease. | 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.<ref name=tt>{{Cite journal | ||
| author = [[Steven Sanchez]] & [[April Ewton]] | |||
| title = Essential thrombocythemia: a review of diagnostic and pathologic features | |||
| journal = [[Archives of pathology & laboratory medicine]] | |||
| volume = 130 | |||
| issue = 8 | |||
| pages = 1144–1150 | |||
| year = 2006 | |||
| month = August | |||
| pmid = 16879015 | |||
}}</ref> Subsequently, essential thrombocytosis was classified as a myeloproliferative disorder along with chronic myelogenous leukemia (CML), polycythemia vera (PV), and chronic idiopathic [[myelofibrosis]] (CIMF).<ref name="LevineGilliland2008">{{cite journal|last1=Levine|first1=R. L.|last2=Gilliland|first2=D. G.|title=Myeloproliferative disorders|journal=Blood|volume=112|issue=6|year=2008|pages=2190–2198|issn=0006-4971|doi=10.1182/blood-2008-03-077966}}</ref> 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.<ref>Platelet. Wikipedia. https://en.wikipedia.org/wiki/Platelet Accessed on November 11, 2015.</ref> 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.<ref name="pmid19636672">{{cite journal| author=Fabris F, Randi ML| title=Essential thrombocythemia: past and present. | journal=Intern Emerg Med | year= 2009 | volume= 4 | issue= 5 | pages= 381-8 | pmid=19636672 | doi=10.1007/s11739-009-0284-x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19636672 }} </ref> The prevalence of essential thrombocytosis is about 30 for every 100,000 people worldwide.<ref name=fm>Essential Thrombocythemia. Genetics Home Reference. http://ghr.nlm.nih.gov/condition/essential-thrombocythemia. Accessed on October 29, 2015</ref> Females are more commonly affected with essential thrombocytosis than males.<ref name=gb>Essential Thrombocythemia (ET). MPN Research foundation. http://www.mpnresearchfoundation.org/Essential-Thrombocythemia Accessed on November 15, 2015.</ref> The female to male ratio is approximately 2 to 1.<ref name="pmid19636672">{{cite journal| author=Fabris F, Randi ML| title=Essential thrombocythemia: past and present. | journal=Intern Emerg Med | year= 2009 | volume= 4 | issue= 5 | pages= 381-8 | pmid=19636672 | doi=10.1007/s11739-009-0284-x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19636672 }} </ref> Symptoms<ref name="pmid17210076">{{cite journal| author=Brière JB| title=Essential thrombocythemia. | journal=Orphanet J Rare Dis | year= 2007 | volume= 2 | issue= | pages= 3 | pmid=17210076 | doi=10.1186/1750-1172-2-3 | pmc=PMC1781427 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17210076 }} </ref> 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.<ref name="pmid17210076">{{cite journal| author=Brière JB| title=Essential thrombocythemia. | journal=Orphanet J Rare Dis | year= 2007 | volume= 2 | issue= | pages= 3 | pmid=17210076 | doi=10.1186/1750-1172-2-3 | pmc=PMC1781427 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17210076 }} </ref> 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== | ==References== | ||
Revision as of 16:22, 17 November 2015
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Essential thrombocytosis Microchapters |
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Differentiating Essential thrombocytosis from other Diseases |
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Essential thrombocytosis overview On the Web |
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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.