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. The disease, also called as Epstein-Goedel syndrome was first described by pathologists Emil Epstein and Alfred Goedel in the year 1934.<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 platelets/ | Essential ([[ET]]) or primary thrombocytosis is a rare hematologic disorder characterized by overproduction of platelets in the absence of an underlying disease. The disease, also called as Epstein-Goedel syndrome was first described by pathologists Emil Epstein and Alfred Goedel in the year 1934.<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. [[Thrombocytosis]] is when the counts stay consistently higher at or more than 150,000-450,000,000 ×10³ platelets/L of blood and [[thrombocytopenia]] is when the counts are consistently lower than 150,000,000 ×10³ platelets/L of blood. Essential thrombocytosis is grouped under the [[myeloproliferative]] disorders along with [[polycythemia vera]], [[chronic myeloid leukaemia]] and [[myelofibrosis]]. Rarely the disease progresses to [[acute myeloid leukemia]] (AML). The diagnosis of essential thrombocytosis is based on the [[WHO]] criteria, which include sustained elevation of platelet count >450,000/μL, [[bone marrow]] biopsy specimen showing proliferation mainly of the megakaryocytic lineage with increased numbers of enlarged, mature [[megakaryocyte]]s; not meeting WHO criteria for [[polycythemia vera]], [[primary myelofibrosis]], BCR-ABL1–positive [[CML]], or [[myelodysplastic syndrome]], or other myeloid neoplasm; and demonstration of ''[[JAK2]] V617F'' or other clonal marker, or in the absence of ''JAK2 V617F'', no evidence of reactive thrombocytosis.<ref name="VardimanThiele2009">{{cite journal|last1=Vardiman|first1=J. W.|last2=Thiele|first2=J.|last3=Arber|first3=D. A.|last4=Brunning|first4=R. D.|last5=Borowitz|first5=M. J.|last6=Porwit|first6=A.|last7=Harris|first7=N. L.|last8=Le Beau|first8=M. M.|last9=Hellstrom-Lindberg|first9=E.|last10=Tefferi|first10=A.|last11=Bloomfield|first11=C. D.|title=The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes|journal=Blood|volume=114|issue=5|year=2009|pages=937–951|issn=0006-4971|doi=10.1182/blood-2009-03-209262}}</ref> | ||
==References== | ==References== | ||
Revision as of 15:54, 17 November 2015
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Essential thrombocytosis Microchapters |
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Differentiating Essential thrombocytosis from other Diseases |
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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 (ET) or primary thrombocytosis is a rare hematologic disorder characterized by overproduction of platelets in the absence of an underlying disease. The disease, also called as Epstein-Goedel syndrome was first described by pathologists Emil Epstein and Alfred Goedel in the year 1934.[1] 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.[2] Normal platelet counts usually range from 150,000-450,000,000 ×10³ platelets/L of blood. Thrombocytosis is when the counts stay consistently higher at or more than 150,000-450,000,000 ×10³ platelets/L of blood and thrombocytopenia is when the counts are consistently lower than 150,000,000 ×10³ platelets/L of blood. Essential thrombocytosis is grouped under the myeloproliferative disorders along with polycythemia vera, chronic myeloid leukaemia and myelofibrosis. Rarely the disease progresses to acute myeloid leukemia (AML). The diagnosis of essential thrombocytosis is based on the WHO criteria, which include sustained elevation of platelet count >450,000/μL, bone marrow biopsy specimen showing proliferation mainly of the megakaryocytic lineage with increased numbers of enlarged, mature megakaryocytes; not meeting WHO criteria for polycythemia vera, primary myelofibrosis, BCR-ABL1–positive CML, or myelodysplastic syndrome, or other myeloid neoplasm; and demonstration of JAK2 V617F or other clonal marker, or in the absence of JAK2 V617F, no evidence of reactive thrombocytosis.[3]
References
- ↑ 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.
- ↑ Vardiman, J. W.; Thiele, J.; Arber, D. A.; Brunning, R. D.; Borowitz, M. J.; Porwit, A.; Harris, N. L.; Le Beau, M. M.; Hellstrom-Lindberg, E.; Tefferi, A.; Bloomfield, C. D. (2009). "The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes". Blood. 114 (5): 937–951. doi:10.1182/blood-2009-03-209262. ISSN 0006-4971.