Table of contents
Essential thrombocythemia
Guidelines
Key sources
The following summarized guidelines for the evaluation and management of essential thrombocythemia are prepared by our editorial team based on guidelines from the European Leukemia Net (ELN 2018), the European Society of Medical Oncology (ESMO 2015), the British Committee for Standards In Haematology (BCSH 2012), and the British Society for Haematology (BSH 2010).
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Screening and diagnosis
Indications for testing: as per BSH 2010 guidelines, evaluate all patients with unexplained splanchnic vein thrombosis for the presence of myeloproliferative neoplasms even if the blood count is normal.
B
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Diagnostic criteria
Classification and risk stratification
Risk of thrombosis
As per ELN 2018 guidelines:
Use the IPSET-thrombosis system (including age, previous thrombosis, cardiovascular risk factors, and JAK2V617F mutation) for prognostic purposes in all patients at diagnosis of ET.
E
Consider assessing general risk factors for thrombosis, including smoking history, diabetes mellitus, hypertension, and hyperlipidemia.
E
Diagnostic investigations
Diagnostic procedures
Medical management
Treatment target: as per ESMO 2015 guidelines, set an optimal platelet target of < 400×10⁹/L (although unvalidated) in patients with ET.
B
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Cytoreductive therapy
Antiplatelet therapy
Perioperative care
Specific circumstances
Pediatric patients
As per BSH 2010 guidelines:
Exclude reactive thrombocytosis rigorously and diagnose ET only in the presence of definitive diagnostic features.
B
Insufficient evidence to guide the management of ET in pediatric patients, however, offer a conservative approach where possible.
I
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Pregnant patients
Breastfeeding patients
Patients on hormonal therapy
Patients undergoing surgery
Patients with leukemic transformation
Patients with post-ET myelofibrosis