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Essential thrombocythemia

Última revisão: 21 Sep 2025
Última atualização: 03 Dec 2024

Resumo

Definition

History and exam

Key diagnostic factors

  • erythromelalgia
  • splenomegaly
  • arterial and venous thrombosis
  • bleeding
  • livedo reticularis
Full details

Other diagnostic factors

  • age ≥60 years
  • female sex
  • headache
  • dizziness, lightheadedness, chest pain, vertigo, and paresthesia
  • syncope and seizures
  • transient visual disturbances
  • priapism
Full details

Risk factors

  • genetic mutations (JAK2 V617F, CALR, or MPL)
  • black ethnicity
  • age ≥60 years
  • female sex
Full details

Diagnostic tests

1st tests to order

  • CBC with differential
  • peripheral blood smear
  • serum iron studies
Full details

Tests to consider

  • CRP
  • erythrocyte sedimentation rate (ESR)
  • fibrinogen
  • bone marrow biopsy and histopathology
  • genetic mutation testing (JAK2 V617F, CALR, and MPL)
  • cytogenetic and molecular testing: BCR::ABL1
Full details

Treatment algorithm

ACUTE

life-threatening thrombosis, severe thrombocytosis-related neurologic complications, or severe bleeding

ONGOING

nonpregnant: very low-, low-, or intermediate-risk

nonpregnant: high risk

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

pregnant: high-risk

Contributors

Authors

Debabrata Mukherjee, MD, FACC
Debabrata Mukherjee

Chairman, Department of Internal 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

Раскрытие информации

TK declares that he has no competing interests.

Uri Rozovski, MD

Department of Cardiology

Tel Aviv Sourasky Medical Center

Tel Aviv

Israel

Раскрытие информации

UR declares that he has no competing interests.

David A. Garcia, MD

Professor

Division of Hematology

University of Washington Seattle

WA

Раскрытие информации

DAG declares that he has no competing interests.

Bethany Samuelson, MD

Fellow

Division of Hematology

University of Washington

Seattle

WA

Раскрытие информации

BS declares that she has no competing interests.

Peer reviewer acknowledgements

BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.

Disclosures

Peer reviewer affiliations and disclosures pertain to the time of the review.

Список литературы

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Основные статьи

National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: myeloproliferative neoplasms [internet publication].Полный текст

Tefferi A, Vannucchi AM, Barbui T. Essential thrombocythemia: 2024 update on diagnosis, risk stratification, and management. Am J Hematol. 2024 Apr;99(4):697-718.Полный текст  Аннотация

Harrison CN, Butt N, Campbell P, et al. Modification of British Committee for Standards in Haematology diagnostic criteria for essential thrombocythaemia. Br J Haematol. 2014 Nov;167(3):421-3. Аннотация

Vannucchi AM, Barbui T, Cervantes F, et al. Philadelphia chromosome-negative chronic myeloproliferative neoplasms: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2015 Sep;26(suppl 5):v85-99.Полный текст  Аннотация

Harrison CN, Bareford D, Butt N, et al; British Committee for Standards in Haematology. Guideline for investigation and management of adults and children presenting with a thrombocytosis. Br J Haematol. 2010 May;149(3):352-75.Полный текст  Аннотация

Статьи, указанные как источники

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