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Subject Area

Pediatrics

Article Type

Review

Abstract

Objective The objective of this study was to review the pathobiology and management of essential thrombocythemia (ET). Materials and methods A systematic search of MEDLINE (PubMed, Medscape, Science Direct, EMF-Portal) and Internet was conducted on all articles published from 1996 to 2017. English language reports of the pathobiology and management of ET were included. The initial search presented 155 articles; of which, 27 satisfied the inclusion criteria. Articles not reporting on the pathobiology and management of ET in the title or abstract were not included. Eleven independent investigators extracted data on methods. Comparisons were made by structured review with the results tabulated. The included articles were eight studies about the pathogenesis of the thrombosis, eight about prognostic factors and risk stratification, and 11 about management of ET. Results The pathogenesis of the thrombosis seen in ET is not fully understood. Advanced age and previous history of thrombosis are the more important determinants of thrombosis. The possible importance in myeloproliferative neoplasm thrombosis of leukocytes and JAK 2 mutational status and allele burden has been a field of intense investigation. Antiplatelet therapy reduced the risk of venous thrombosis in JAK2-positive patients and the risk of arterial thrombosis in those with cardiovascular risk factors, whereas it was associated with an excess of bleeding episodes in patients with platelet counts above 1000 × 109/l. Conclusion ET is a chronic disease that has no cure. It may not need treatment in a mild condition, whereas in severe condition, it may need medicine that lowers platelet count, blood thinners, or both. Treatment is controversial but may include aspirin.

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