Essential thrombocytosis

Last reviewed: 7 Nov 2022
Last updated: 23 Nov 2021

Summary

Definition

History and exam

Key diagnostic factors

  • erythromelalgia
  • splenomegaly
  • arterial and venous thrombosis
  • bleeding
  • livedo reticularis
More key diagnostic factors

Other diagnostic factors

  • age 50 to 70 years
  • female sex
  • no symptoms
  • headache
  • dizziness, lightheadedness, and paraesthesias
  • syncope and seizures
  • transient visual disturbances
  • hepatomegaly
  • priapism
Other diagnostic factors

Risk factors

  • Unknown risk factors
  • Genetic mutations (e.g., JAK2, CALR, or MPL)
More risk factors

Diagnostic investigations

1st investigations to order

  • FBC with peripheral smear
  • iron panel
More 1st investigations to order

Investigations to consider

  • CRP
  • ESR
  • fibrinogen
  • bone marrow biopsy and histopathology
  • Janus kinase 2 (JAK2) mutation testing of peripheral blood
  • myeloproliferative leukaemia virus oncogene (MPL) mutation testing of peripheral blood
  • calreticulin (CALR) mutation testing of peripheral blood
  • BCR-ABL fluorescent in situ hybridisation (FISH) of bone marrow
More investigations to consider

Treatment algorithm

ACUTE

thrombosis or bleeding

ONGOING

non-pregnant: very low-, low-, or intermediate-risk

non-pregnant: high risk

pregnant: very low- or low-risk

pregnant: intermediate-risk

pregnant: high-risk

Contributors

Authors

Debabrata Mukherjee, MD, FACC
Debabrata Mukherjee

Chairman, Department of Internal Medicine

Chief, Cardiovascular Medicine

Professor of Internal Medicine

Texas Tech University Health Sciences Center at El Paso

El Paso

TX

Disclosures

DM declares that he has no competing interests.

Peer reviewers

Tony Kan, MD

Senior Staff Physician

Henry Ford Medical Center

Sterling Heights

MI

Disclosures

TK declares that he has no competing interests.

Uri Rozovski, MD

Department of Cardiology

Tel Aviv Sourasky Medical Center

Tel Aviv

Israel

Disclosures

UR declares that he has no competing interests.

David A. Garcia, MD

Professor

Division of Hematology

University of Washington Seattle

WA

Disclosures

DAG declares that he has no competing interests.

Bethany Samuelson, MD

Fellow

Division of Hematology

University of Washington

Seattle

WA

Disclosures

BS declares that she has no competing interests.

  • Essential thrombocytosis images
  • Differentials

    • Secondary (reactive) thrombocytosis
    • Spurious thrombocytosis
    • Familial essential thrombocythaemia
    More Differentials
  • Guidelines

    • Unmet clinical needs in the management of CALR-mutated essential thrombocythaemia: a consensus-based proposal from the European LeukemiaNet
    • Essential thrombocythemia treatment algorithm 2018
    More Guidelines
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