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==Overview==
==Overview==
If left untreated, patients with essential thrombocytosis  may progress to develop symptoms like headache, dizziness, vision disturbances, chest pain, intense burning pain in hands and/or feet ([[erythromelalgia]]), numbness and tingling of hands and feet, [[priapism]] (persistent and painful erection of the penis) and so on depending on the vessel occluded with the thrombus. Complications that can develop from essential thrombocytosis are thrombotic events ([[DVT]], cerebrovascular accidents,etc), bleeding (bruises, gum bleeds, [[epistaxis]], etc), [[acute leukemia]] and [[myelofibrosis]]. Essential thrombocytosis is associated with a good prognosis if patients are medically supervised on a regular basis. Lifespan is usually normal as any other unaffected person, though some patients may have symptoms from vascular occlusion and/or bleeding. Rarely the disease may undergo a leukemic conversion or [[myelofibrosis]].   
If left untreated, patients with essential thrombocytosis  may progress to develop symptoms like headache, [[dizziness]], vision disturbances, chest pain, intense burning pain in hands and/or feet ([[erythromelalgia]]), numbness and tingling of hands and feet, and [[priapism]] (persistent and painful erection of the penis) depending on the vessel occluded with the thrombus. Common complications of essential thrombocytosis include thrombotic events ([[DVT]], cerebrovascular accidents,etc), bleeding (bruises, gum bleeds, [[epistaxis]], etc), [[acute leukemia]], and [[myelofibrosis]].<ref name = paed>{{cite journal|last=Frewin|first=R|coauthors=Dowson, A|title=Headache in essential thrombocythaemia.|journal=International Journal of Clinical Practice|date=October 2012|volume=66|issue=10|pages=976-83|doi=10.1111/j.1742-1241.2012.02986.x|pmid=22889110|url=http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3469735/pdf/ijcp0066-0976.pdf|format=PDF|pmc=3469735}}</ref><ref>{{cite journal|last=Tefferi|first=A|title=Annual Clinical Updates in Hematological Malignancies: a continuing medical education series: polycythemia vera and essential thrombocythemia: 2011 update on diagnosis, risk-stratification, and management.|journal=American Journal of Hematology|date=March 2011|volume=86|issue=3|pages=292-301|doi=10.1002/ajh.21946|pmid=21351120}}</ref> Prognosis is generally good, and the survival rate of patients is usually normal with  regular medical supervision. However, the patient may rarely experience a leukemic conversion or develop [[myelofibrosis]].   


==Natural history==
==Natural history==
The symptoms of essential thrombocytosis usually develop in two peaks, one in the third decade of life, and the other in the sixth decade of life. It may present with symptoms including but not limited to headache, dizziness, vision disturbances, chest pain, intense burning pain in hands and/or feet ([[erythromelalgia]]), numbness and tingling of hands and feet and [[priapism]] (persistent and painful erection of the penis). Without treatment, patients with very high counts will develop serious complications from vascular occlusion and/or bleeding. Rarely some cases progress to fatal leukemia or [[myelofibrosis]].  
*The majority of patients with essential thrombocytosis remain asymptomatic for a long time and are diagnosed on routine blood tests or when tested for other conditions. Although some patients with very high counts may present with severe hemorrhagic or thombotic events, needing treatment.
*The symptoms arise from complications of essential thrombocytosis and may include but are not limited to headache, dizziness, vision disturbances, chest pain, intense burning pain in hands and/or feet ([[erythromelalgia]]), numbness and tingling of hands and feet and [[priapism]] (persistent and painful erection of the penis).<ref name = paed/><ref>{{cite journal|last=Frewin|first=R|coauthors=Dowson, A|title=Headache in essential thrombocythaemia.|journal=International Journal of Clinical Practice|date=October 2012|volume=66|issue=10|pages=976-83|doi=10.1111/j.1742-1241.2012.02986.x|pmid=22889110|url=http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3469735/pdf/ijcp0066-0976.pdf|format=PDF|pmc=3469735}}</ref><ref>{{cite journal|last=Tefferi|first=A|title=Annual Clinical Updates in Hematological Malignancies: a continuing medical education series: polycythemia vera and essential thrombocythemia: 2011 update on diagnosis, risk-stratification, and management.|journal=American Journal of Hematology|date=March 2011|volume=86|issue=3|pages=292-301|doi=10.1002/ajh.21946|pmid=21351120}}</ref>
*In predisposed individuals, gastrointestinal bleeding may sometimes worsen with [[aspirin]] where it is used to relieve symptoms of mild essential thrombocytosis. Hence it is contraindicated in these patients.<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>
*Without treatment, patients with very high platelet counts will develop serious complications from vascular occlusion and/or bleeding.  
*Rarely some cases progress to fatal leukemia or may develop [[myelofibrosis]].  


==Complications==
==Complications==
*Some of the complications from essential thrombocytosis are as follows:
*Some of the complications from essential thrombocytosis are as follows<ref name = paed/><ref>{{cite journal|last=Frewin|first=R|coauthors=Dowson, A|title=Headache in essential thrombocythaemia.|journal=International Journal of Clinical Practice|date=October 2012|volume=66|issue=10|pages=976-83|doi=10.1111/j.1742-1241.2012.02986.x|pmid=22889110|url=http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3469735/pdf/ijcp0066-0976.pdf|format=PDF|pmc=3469735}}</ref><ref>{{cite journal|last=Tefferi|first=A|title=Annual Clinical Updates in Hematological Malignancies: a continuing medical education series: polycythemia vera and essential thrombocythemia: 2011 update on diagnosis, risk-stratification, and management.|journal=American Journal of Hematology|date=March 2011|volume=86|issue=3|pages=292-301|doi=10.1002/ajh.21946|pmid=21351120}}</ref>:
**Thrombotic events  
**Thrombotic events  
***[[Pulmonary embolism]]
***[[DVT]]
***[[DVT]]
***Cerebrovascular accidents ([[CVA]])
***Cerebrovascular accidents ([[CVA]])
Line 21: Line 26:
**[[Acute leukemia]]
**[[Acute leukemia]]
**[[Myelofibrosis]]
**[[Myelofibrosis]]
*In predisposed individuals, gastrointestinal bleeding may sometimes worsen with [[aspirin]] where it is used to relieve symptoms of mild ET. Hence it is contraindicated in these patients.<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>
*Complications may occur in pregnant patients with essential thrombocytosis. High platelet counts cause placental [[infarction]] and places the [[fetus]] at a higher risk than the mother. The complications may include the following:
*Complications may occur in pregnant ET patients. High platelet counts cause placental infarcts and places the fetus at a higher risk than the mother. The complications may include the following:
**Increased risk of thrombosis
**Increased risk of thrombosis
**Increased risk of spontaneous miscarriage
**Increased risk of spontaneous miscarriage
**IUGR
**[[IUGR]]
**Preterm labor
**Preterm labor
**Placental abruption
**[[Placental abruption]]


==Prognosis==
==Prognosis==
*Essential thrombocytosis is associated with a good prognosis if medically supervised on a regular basis.   
*Essential thrombocytosis is associated with a good prognosis if medically supervised on a regular basis.   
*Most of the patients remain asymptomatic for a long time and are diagnosed on routine blood tests or when tested for other conditions. Although some patients with very high counts may present with severe hemorrhagic or thombotic events needing treatment.  
*Pregnancy in patients with essential thrombocytosis is associated with a two to three fold increase in risk for spontaneous micarriage and need special care.<ref>Essential thrombocythemia. Orphanet journal of rare diseases. http://www.ojrd.com/content/2/1/3 Accessed on November 11, 2015.</ref>
*Rarely the disease may progress to [[acute leukemia]] or [[myelofibrosis]].


==References==
==References==

Latest revision as of 16:01, 6 January 2016

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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

If left untreated, patients with essential thrombocytosis may progress to develop symptoms like headache, dizziness, vision disturbances, chest pain, intense burning pain in hands and/or feet (erythromelalgia), numbness and tingling of hands and feet, and priapism (persistent and painful erection of the penis) depending on the vessel occluded with the thrombus. Common complications of essential thrombocytosis include thrombotic events (DVT, cerebrovascular accidents,etc), bleeding (bruises, gum bleeds, epistaxis, etc), acute leukemia, and myelofibrosis.[1][2] Prognosis is generally good, and the survival rate of patients is usually normal with regular medical supervision. However, the patient may rarely experience a leukemic conversion or develop myelofibrosis.

Natural history

  • The majority of patients with essential thrombocytosis remain asymptomatic for a long time and are diagnosed on routine blood tests or when tested for other conditions. Although some patients with very high counts may present with severe hemorrhagic or thombotic events, needing treatment.
  • The symptoms arise from complications of essential thrombocytosis and may include but are not limited to headache, dizziness, vision disturbances, chest pain, intense burning pain in hands and/or feet (erythromelalgia), numbness and tingling of hands and feet and priapism (persistent and painful erection of the penis).[1][3][4]
  • In predisposed individuals, gastrointestinal bleeding may sometimes worsen with aspirin where it is used to relieve symptoms of mild essential thrombocytosis. Hence it is contraindicated in these patients.[5]
  • Without treatment, patients with very high platelet counts will develop serious complications from vascular occlusion and/or bleeding.
  • Rarely some cases progress to fatal leukemia or may develop myelofibrosis.

Complications

Prognosis

  • Essential thrombocytosis is associated with a good prognosis if medically supervised on a regular basis.
  • Pregnancy in patients with essential thrombocytosis is associated with a two to three fold increase in risk for spontaneous micarriage and need special care.[8]

References

  1. ↑ 1.0 1.1 1.2 Frewin, R (October 2012). "Headache in essential thrombocythaemia" (PDF). International Journal of Clinical Practice. 66 (10): 976–83. doi:10.1111/j.1742-1241.2012.02986.x. PMC 3469735. PMID 22889110. Unknown parameter |coauthors= ignored (help)
  2. ↑ Tefferi, A (March 2011). "Annual Clinical Updates in Hematological Malignancies: a continuing medical education series: polycythemia vera and essential thrombocythemia: 2011 update on diagnosis, risk-stratification, and management". American Journal of Hematology. 86 (3): 292–301. doi:10.1002/ajh.21946. PMID 21351120.
  3. ↑ Frewin, R (October 2012). "Headache in essential thrombocythaemia" (PDF). International Journal of Clinical Practice. 66 (10): 976–83. doi:10.1111/j.1742-1241.2012.02986.x. PMC 3469735. PMID 22889110. Unknown parameter |coauthors= ignored (help)
  4. ↑ Tefferi, A (March 2011). "Annual Clinical Updates in Hematological Malignancies: a continuing medical education series: polycythemia vera and essential thrombocythemia: 2011 update on diagnosis, risk-stratification, and management". American Journal of Hematology. 86 (3): 292–301. doi:10.1002/ajh.21946. PMID 21351120.
  5. ↑ Brière JB (2007). "Essential thrombocythemia". Orphanet J Rare Dis. 2: 3. doi:10.1186/1750-1172-2-3. PMC 1781427. PMID 17210076.
  6. ↑ Frewin, R (October 2012). "Headache in essential thrombocythaemia" (PDF). International Journal of Clinical Practice. 66 (10): 976–83. doi:10.1111/j.1742-1241.2012.02986.x. PMC 3469735. PMID 22889110. Unknown parameter |coauthors= ignored (help)
  7. ↑ Tefferi, A (March 2011). "Annual Clinical Updates in Hematological Malignancies: a continuing medical education series: polycythemia vera and essential thrombocythemia: 2011 update on diagnosis, risk-stratification, and management". American Journal of Hematology. 86 (3): 292–301. doi:10.1002/ajh.21946. PMID 21351120.
  8. ↑ Essential thrombocythemia. Orphanet journal of rare diseases. http://www.ojrd.com/content/2/1/3 Accessed on November 11, 2015.


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