Resumo
Definição
História e exame físico
Principais fatores diagnósticos
- erythromelalgia
- splenomegaly
- arterial and venous thrombosis
- bleeding
- livedo reticularis
Outros fatores diagnósticos
- age ≥60 years
- female sex
- headache
- dizziness, lightheadedness, chest pain, vertigo, and paresthesia
- syncope and seizures
- transient visual disturbances
- priapism
Fatores de risco
- genetic mutations (JAK2 V617F, CALR, or MPL)
- black ethnicity
- age ≥60 years
- female sex
Investigações diagnósticas
Primeiras investigações a serem solicitadas
- CBC with differential
- peripheral blood smear
- serum iron studies
Investigações a serem consideradas
- 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
Algoritmo de tratamento
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
Colaboradores
Autores
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
Declarações
DM declares that he has no competing interests.
Revisores
Tony Kan, MD
Senior Staff Physician
Henry Ford Medical Center
Sterling Heights
MI
Declarações
TK declares that he has no competing interests.
Uri Rozovski, MD
Department of Cardiology
Tel Aviv Sourasky Medical Center
Tel Aviv
Israel
利益声明
UR declares that he has no competing interests.
David A. Garcia, MD
Professor
Division of Hematology
University of Washington Seattle
WA
利益声明
DAG declares that he has no competing interests.
Bethany Samuelson, MD
Fellow
Division of Hematology
University of Washington
Seattle
WA
利益声明
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.
参考文献
关键文献
National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: myeloproliferative neoplasms [internet publication].全文
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.全文 摘要
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. 摘要
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.全文 摘要
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.全文 摘要
参考文献
A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
鉴别诊断
- Secondary (reactive) thrombocytosis
- Spurious thrombocytosis
- Familial essential thrombocythemia
更多 鉴别诊断指南
- 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
更多 指南
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