Summary
Definition
History and exam
Key diagnostic factors
- erythromelalgia
- splenomegaly
- arterial and venous thrombosis
- bleeding
- livedo reticularis
Other diagnostic factors
- age ≥60 years
- female sex
- headache
- dizziness, lightheadedness, chest pain, vertigo, and paresthesia
- syncope and seizures
- transient visual disturbances
- priapism
Risk factors
- genetic mutations (JAK2 V617F, CALR, or MPL)
- black ethnicity
- age ≥60 years
- female sex
Diagnostic investigations
1st investigations to order
- CBC with differential
- peripheral blood smear
- serum iron studies
Investigations 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
Treatment algorithm
life-threatening thrombosis, severe thrombocytosis-related neurologic complications, or severe bleeding
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
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
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
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.
Differentials
- Secondary (reactive) thrombocytosis
- Spurious thrombocytosis
- Familial essential thrombocythemia
More DifferentialsGuidelines
- 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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