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Essential thrombocythemia


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.
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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.
Consider assessing general risk factors for thrombosis, including smoking history, diabetes mellitus, hypertension, and hyperlipidemia.

Diagnostic investigations

Genetic testing: as per ELN 2018 guidelines, obtain mutational analysis for JAK2V617F, CALR, and MPL on peripheral blood or bone marrow samples in patients with suspected ET.

Diagnostic procedures

Bone marrow biopsy: as per ELN 2018 guidelines, perform bone marrow biopsy in all patients with suspected Philadelphia chromosome-negative myeloproliferative neoplasms, except for patients with polycythemia vera with hemoglobin > 18.5 g/dL in males and > 16.5 g/dL in females.

Medical management

Treatment target: as per ESMO 2015 guidelines, set an optimal platelet target of < 400×10⁹/L (although unvalidated) in patients with ET.

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  • Cytoreductive therapy

  • Antiplatelet therapy

Perioperative care

Preoperative management: as per ESMO 2015 guidelines, consider controlling blood counts preoperatively to standard targets in high-risk patients undergoing surgery, when bleeding is a risk or thromboprophylaxis would normally be prescribed.

Specific circumstances

Pediatric patients
As per BSH 2010 guidelines:
Exclude reactive thrombocytosis rigorously and diagnose ET only in the presence of definitive diagnostic features.
Insufficient evidence to guide the management of ET in pediatric patients, however, offer a conservative approach where possible.

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  • Pregnant patients

  • Breastfeeding patients

  • Patients on hormonal therapy

  • Patients undergoing surgery

  • Patients with leukemic transformation

  • Patients with post-ET myelofibrosis