Abstract:
:In the past 5 years we have witnessed significant advances in both the diagnostic process and optimal therapy for patients with essential thrombocythemia (ET). Insights into the underlying molecular mechanisms have been accompanied by the development of new diagnostic tests and by an improved understanding of the relationship between ET and other related myeloproliferative neoplasms, such as polycythemia vera and primary myelofibrosis. In the first part of this review, we describe how recent molecular and histologic studies can be integrated into a streamlined diagnostic process that is applicable to everyday clinical practice. We also address areas of current diagnostic controversy, including heterogeneity within ET and the phenotypic overlap between ET, polycythemia vera, and primary myelofibrosis. In the second part, we provide an overview of our current approach to the treatment of ET, including risk stratification, choice of cytoreductive agent, and a consideration of special situations such as the pregnant or perioperative patient. Areas of controversy discussed include the identification of those at high risk of complications and therapeutic decisions in the younger patient.
journal_name
Bloodjournal_title
Bloodauthors
Beer PA,Erber WN,Campbell PJ,Green ARdoi
10.1182/blood-2010-08-270033subject
Has Abstractpub_date
2011-02-03 00:00:00pages
1472-82issue
5eissn
0006-4971issn
1528-0020pii
blood-2010-08-270033journal_volume
117pub_type
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