Essential thrombocytosis medical therapy: Difference between revisions

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==Risk Stratification==
==Risk Stratification==
==Medical therapy==
*Individuals with essential thrombocytosis are grouped into high-risk and low-risk groups, based on the presence or absence of risk factors for the development of complications.<ref name="pmid21106990">{{cite journal| author=Beer PA, Erber WN, Campbell PJ, Green AR| title=How I treat essential thrombocythemia. | journal=Blood | year= 2011 | volume= 117 | issue= 5 | pages= 1472-82 | pmid=21106990 | doi=10.1182/blood-2010-08-270033 | pmc=PMC3145107 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21106990  }} </ref>
*Risk factors guide in the therapeutic decisions.
*The risk factors for complications include:<ref name="pmid21106990">{{cite journal| author=Beer PA, Erber WN, Campbell PJ, Green AR| title=How I treat essential thrombocythemia. | journal=Blood | year= 2011 | volume= 117 | issue= 5 | pages= 1472-82 | pmid=21106990 | doi=10.1182/blood-2010-08-270033 | pmc=PMC3145107 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21106990  }} </ref>
 
{| style="border: 0px; font-size: 90%; margin: 3px; width:650px"
|valign=top|
|+
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|High risk}}
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|Intermediate risk}}
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|Low risk}}
 
|-
| style="padding: 5px 5px; background: #F5F5F5;"  align=left|
*Age > 60 years
:
*History of thrombotic events
:
*Platelet count >1500 × 10<sup>9</sup>/L
 
| style="padding: 5px 5px; background: #F5F5F5;"  align=center|
:Age 40-60 years
 
| style="padding: 5px 5px; background: #F5F5F5;"  align=center|
:Age < 40 years
 
|}
 
==Medical Therapy==
*Majority of the patients with essential thrombocytosis are asymptomatic and do not require treatment. Patients are usually diagnosed with essential thrombocytosis on routine testing for other conditions.
*Majority of the patients with essential thrombocytosis are asymptomatic and do not require treatment. Patients are usually diagnosed with essential thrombocytosis on routine testing for other conditions.
*Low risk patients are placed on low dose [[aspirin]] therapy to lower the risk of [[thrombosis]], but there may be an increased risk of bleeding if aspirin is initiated whilst the platelet count is very high or if the patient is predisposed to gastrointestinal bleeds.<ref>Prognosis and treatment of essential thrombocythemia. UpToDate.http://www.uptodate.com/contents/prognosis-and-treatment-of-essential-thrombocythemia Accessed on November 12, 2015.</ref> In the latter cases, aspirin is withheld.  
*Low risk patients are placed on low dose [[aspirin]] therapy to lower the risk of [[thrombosis]], but there may be an increased risk of bleeding if aspirin is initiated whilst the platelet count is very high or if the patient is predisposed to gastrointestinal bleeds.<ref>Prognosis and treatment of essential thrombocythemia. UpToDate.http://www.uptodate.com/contents/prognosis-and-treatment-of-essential-thrombocythemia Accessed on November 12, 2015.</ref> In the latter cases, aspirin is withheld.  
*In those who are at increased risk of thrombosis or bleeding, reduction of the platelet count to the normal range can be achieved using [[hydroxyurea]] ([[hydroxycarbamide]]), [[interferon-α]] or [[anagrelide]]. Old age, previous history of bleeding or thrombosis, or very high platelet count are considered to be high risk factors.  
*In those who are at increased risk of thrombosis or bleeding, reduction of the platelet count to the normal range can be achieved using [[hydroxyurea]] ([[hydroxycarbamide]]), [[interferon-α]] or [[anagrelide]]. Old age, previous history of bleeding or thrombosis, or very high platelet count are considered to be high risk factors.  
*The PT1 study <ref name=Harrison>Harrison CN et al. ''Hydroxyurea compared with anagrelide in high-risk essential thrombocythemia.'' N Engl J Med. 2005;7:33-45. PMID 16000354.</ref> compared hydroxyurea in combination with aspirin to anagrelide in combination with Aspirin as initial therapy for essential thrombocytosis.  Hydroxyurea was superior, with lower risk of arterial thrombosis, lower risk of severe bleeding and lower risk of transformation to myelofibrosis (although the rate of venous thrombosis was higher with hydroxycarbamide than with anagrelide).
*The PT1 study compared hydroxyurea in combination with aspirin to [[anagrelide]] in combination with aspirin as initial therapy for essential thrombocytosis.  [[Hydroxyurea]] was superior, with lower risk of arterial thrombosis, lower risk of severe bleeding and lower risk of transformation to [[myelofibrosis]] (although the rate of venous thrombosis was higher with hydroxycarbamide than with anagrelide).<ref name=Harrison>Harrison CN et al. ''Hydroxyurea compared with anagrelide in high-risk essential thrombocythemia.'' N Engl J Med. 2005;7:33-45. PMID 16000354.</ref>
*In life threatening cases, emergent [[plateletpheresis]] may be performed where the blood of a patient is passed through an apparatus that separates out one particular constituent, platelets in this case and returns the remainder to the circulation.<ref>Plateletpheresis. Wikipedia. https://en.wikipedia.org/wiki/Plateletpheresis Accessed on November 12, 2015.</ref>
*In life threatening cases, emergent [[plateletpheresis]] may be performed where the blood of a patient is passed through an apparatus that separates out one particular constituent, platelets in this case and returns the remainder to the circulation.<ref>Plateletpheresis. Wikipedia. https://en.wikipedia.org/wiki/Plateletpheresis Accessed on November 12, 2015.</ref>
*Special care related to pregnancy:
 
**Pregnancy in patients with essential thrombocytosis is associated with a two to three fold increase in risk for spontaneous micarriage.
==Special Care Related to Pregnancy==
**Hydroxyurea and [[anagrelide]] are contraindicated during [[pregnancy]] and [[nursing]].<ref>{{Cite journal
*Pregnancy in patients with essential thrombocytosis is associated with a two to three fold increase in risk for spontaneous micarriage.
*Hydroxyurea and [[anagrelide]] are contraindicated during [[pregnancy]] and [[nursing]].<ref>{{Cite journal
| author = [[Marie-Cecile Valera]], [[Olivier Parant]], [[Christophe Vayssiere]], [[Jean-Francois Arnal]] & [[Bernard Payrastre]]
| author = [[Marie-Cecile Valera]], [[Olivier Parant]], [[Christophe Vayssiere]], [[Jean-Francois Arnal]] & [[Bernard Payrastre]]
  | title = Essential thrombocythemia and pregnancy
  | title = Essential thrombocythemia and pregnancy
Line 28: Line 56:


}}</ref>
}}</ref>
**Essential thrombocytosis can be linked with increased risk of spontaneous abortion or miscarriage in the first trimester of pregnancy.  Throughout pregnancy, close monitoring of the mother for thrombosis as well as placenta is recommended to ensure blood clots are diagnosed in time for interventions.   
*Essential thrombocytosis can be linked with increased risk of spontaneous abortion or miscarriage in the first trimester of pregnancy.  Throughout pregnancy, close monitoring of the mother for thrombosis as well as placenta is recommended to ensure blood clots are diagnosed in time for interventions.   
**Post partum, often daily injections of low dose [[low molecular weight heparin]] (e.g. [[enoxaparin]]) and low dose aspirin are prescribed as prophylaxis for several weeks as this is a period where the mother is at higher risk of developing a blood clot.<ref>Essential thrombocythemia. Orphanet journal of rare diseases. http://www.ojrd.com/content/2/1/3 Accessed on November 11, 2015.</ref>
*Post partum, often daily injections of low dose [[low molecular weight heparin]] (e.g. [[enoxaparin]]) and low dose aspirin are prescribed as prophylaxis for several weeks as this is a period where the mother is at higher risk of developing a blood clot.<ref>Essential thrombocythemia. Orphanet journal of rare diseases. http://www.ojrd.com/content/2/1/3 Accessed on November 11, 2015.</ref>


==References==
==References==

Revision as of 14:04, 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

The majority of cases of essential thrombocytosis only require supportive care. Anti-thrombotic therapy is individualized based on patients' risk of developing thrombosis, which include aspirin therapy for low-risk patients and platelet lowering drugs (hydroxyurea, interferon-α and anagrelide) for high-risk patients.

Risk Stratification

  • Individuals with essential thrombocytosis are grouped into high-risk and low-risk groups, based on the presence or absence of risk factors for the development of complications.[1]
  • Risk factors guide in the therapeutic decisions.
  • The risk factors for complications include:[1]
High risk Intermediate risk Low risk
  • Age > 60 years
  • History of thrombotic events
  • Platelet count >1500 × 109/L
Age 40-60 years
Age < 40 years

Medical Therapy

  • Majority of the patients with essential thrombocytosis are asymptomatic and do not require treatment. Patients are usually diagnosed with essential thrombocytosis on routine testing for other conditions.
  • Low risk patients are placed on low dose aspirin therapy to lower the risk of thrombosis, but there may be an increased risk of bleeding if aspirin is initiated whilst the platelet count is very high or if the patient is predisposed to gastrointestinal bleeds.[2] In the latter cases, aspirin is withheld.
  • In those who are at increased risk of thrombosis or bleeding, reduction of the platelet count to the normal range can be achieved using hydroxyurea (hydroxycarbamide), interferon-α or anagrelide. Old age, previous history of bleeding or thrombosis, or very high platelet count are considered to be high risk factors.
  • The PT1 study compared hydroxyurea in combination with aspirin to anagrelide in combination with aspirin as initial therapy for essential thrombocytosis. Hydroxyurea was superior, with lower risk of arterial thrombosis, lower risk of severe bleeding and lower risk of transformation to myelofibrosis (although the rate of venous thrombosis was higher with hydroxycarbamide than with anagrelide).[3]
  • In life threatening cases, emergent plateletpheresis may be performed where the blood of a patient is passed through an apparatus that separates out one particular constituent, platelets in this case and returns the remainder to the circulation.[4]

Special Care Related to Pregnancy

  • Pregnancy in patients with essential thrombocytosis is associated with a two to three fold increase in risk for spontaneous micarriage.
  • Hydroxyurea and anagrelide are contraindicated during pregnancy and nursing.[5]
  • Essential thrombocytosis can be linked with increased risk of spontaneous abortion or miscarriage in the first trimester of pregnancy. Throughout pregnancy, close monitoring of the mother for thrombosis as well as placenta is recommended to ensure blood clots are diagnosed in time for interventions.
  • Post partum, often daily injections of low dose low molecular weight heparin (e.g. enoxaparin) and low dose aspirin are prescribed as prophylaxis for several weeks as this is a period where the mother is at higher risk of developing a blood clot.[6]

References

  1. 1.0 1.1 Beer PA, Erber WN, Campbell PJ, Green AR (2011). "How I treat essential thrombocythemia". Blood. 117 (5): 1472–82. doi:10.1182/blood-2010-08-270033. PMC 3145107. PMID 21106990.
  2. Prognosis and treatment of essential thrombocythemia. UpToDate.http://www.uptodate.com/contents/prognosis-and-treatment-of-essential-thrombocythemia Accessed on November 12, 2015.
  3. Harrison CN et al. Hydroxyurea compared with anagrelide in high-risk essential thrombocythemia. N Engl J Med. 2005;7:33-45. PMID 16000354.
  4. Plateletpheresis. Wikipedia. https://en.wikipedia.org/wiki/Plateletpheresis Accessed on November 12, 2015.
  5. Marie-Cecile Valera, Olivier Parant, Christophe Vayssiere, Jean-Francois Arnal & Bernard Payrastre (2011). "Essential thrombocythemia and pregnancy". European journal of obstetrics, gynecology, and reproductive biology. 158 (2): 141–147. doi:10.1016/j.ejogrb.2011.04.040. PMID 21640467. Unknown parameter |month= ignored (help)
  6. Essential thrombocythemia. Orphanet journal of rare diseases. http://www.ojrd.com/content/2/1/3 Accessed on November 11, 2015.


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