Essential thrombocytosis natural history, complications and prognosis: Difference between revisions

Jump to navigation Jump to search
No edit summary
No edit summary
 
Line 7: Line 7:


==Natural history==
==Natural history==
*Essential thrombocytosis is usually asymptomatic.  
*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>  
*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.  
*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]].  
*Rarely some cases progress to fatal leukemia or may develop [[myelofibrosis]].  
Line 15: Line 16:
*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>:
*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
***[[Pulmonary embolism]]
***[[DVT]]
***[[DVT]]
***Cerebrovascular accidents ([[CVA]])
***Cerebrovascular accidents ([[CVA]])
Line 34: Line 35:
==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

Essential thrombocytosis Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Epidemiology and Demographics

Risk Factors

Screening

Causes

Differentiating Essential thrombocytosis from other Diseases

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Criteria

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

Chest X Ray

CT

MRI

Echocardiography or Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Essential thrombocytosis natural history, complications and prognosis On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Essential thrombocytosis natural history, complications and prognosis

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Essential thrombocytosis natural history, complications and prognosis

CDC on Essential thrombocytosis natural history, complications and prognosis

Essential thrombocytosis natural history, complications and prognosis in the news

Blogs on Essential thrombocytosis natural history, complications and prognosis

Directions to Hospitals Treating Essential thrombocytosis

Risk calculators and risk factors for Essential thrombocytosis natural history, complications and prognosis

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.


Template:WikiDoc Sources