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Essential thrombocythemia

Последний просмотренный: 18 Sep 2025
Last updated: 03 Dec 2024

Резюме

Определение

Анамнез и осмотр

Ключевые диагностические факторы

  • erythromelalgia
  • splenomegaly
  • arterial and venous thrombosis
  • bleeding
  • livedo reticularis
Полная информация

Другие диагностические факторы

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

Факторы риска

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

Диагностические исследования

Исследования, которые показаны в первую очередь

  • CBC with differential
  • peripheral blood smear
  • serum iron studies
Полная информация

Исследования, проведение которых нужно рассмотреть

  • 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
Полная информация

Алгоритм лечения

Острый

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

ПРОДОЛЖЕНИЕ

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

nonpregnant: high risk

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

pregnant: high-risk

Составители

Авторы

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

Раскрытие информации

DM declares that he has no competing interests.

Рецензенты

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.

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.

References

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

Key articles

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

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.Full text  Abstract

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. Abstract

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.Full text  Abstract

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.Full text  Abstract

Reference articles

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
  • Essential thrombocythemia images
  • Differentials

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

    • NCCN clinical practice guidelines in oncology: myeloproliferative neoplasms
    • The 5th edition of the World Health Organization (WHO) classification of haematolymphoid tumours: myeloid and histiocytic/dendritic neoplasms
    More Guidelines
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