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Essential thrombocytosis natural history, complications and prognosis

Introduction

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, 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

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

Complications

Prognosis

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

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. PMCΒ 3469735. PMIDΒ 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. PMIDΒ 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. PMCΒ 3469735. PMIDΒ 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. PMIDΒ 21351120.
  5. ↑ BriΓ¨re JB (2007). “Essential thrombocythemia”. Orphanet J Rare Dis. 2: 3. doi:10.1186/1750-1172-2-3. PMCΒ 1781427. PMIDΒ 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. PMCΒ 3469735. PMIDΒ 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. PMIDΒ 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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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Soujanya Thummathati, MBBS [2]