Summary
Definition
History and exam
Key diagnostic factors
- history of radiation exposure
- history of industrial solvents exposure
- symptoms of anemia (fatigue, weakness, dyspnea, palpitations)
- constitutional symptoms (weight loss, night sweats, low-grade fever, cachexia, fatigue, and pruritus)
- splenomegaly ± hepatomegaly
- features of extramedullary hematopoiesis
Other diagnostic factors
- features of portal hypertension
- joint and bone pain
- hearing loss
- bleeding
- infections
Risk factors
- radiation exposure
- industrial solvents exposure
- age ≥65 years
- cytogenetic abnormalities
Diagnostic tests
1st tests to order
- CBC with differential
- peripheral blood smear
- bone marrow aspiration
- bone marrow biopsy
- fluorescence in situ hybridization (FISH) or multiplex reverse transcriptase PCR
- genetic mutation analysis
Tests to consider
- bone marrow cytogenetic analysis
- echocardiogram
- ultrasound of suspected site
- technetium 99 scan
- CT of suspected site
- MRI of suspected site
- serum uric acid
- antinuclear antibodies
- rheumatoid factor titer
- complement levels
- Coombs test
治疗流程
lower risk: asymptomatic
lower risk: symptomatic
higher risk: younger stem cell transplant candidate without comorbidities
higher risk: stem cell transplant candidate >70 years or younger stem cell transplant candidate with comorbidities
higher risk: not stem cell transplant candidate
撰稿人
作者
Jerry L. Spivak, MD

Professor of Medicine and Oncology
Division of Hematology
Johns Hopkins University School of Medicine
Baltimore
MD
利益声明
JLS is an author of several references cited in this topic and has been reimbursed by GSK for a consultation.
鸣谢
Professor Jerry Spivak would like to gratefully acknowledge Dr Ashkan Emadi, a previous contributor to this topic.
利益声明
AE declares that he has no competing interests.
同行评议者
John T. Reilly, BSc, MD, FRCP, FRCPATH
Professor and Consultant in Haematology
Royal Hallamshire Hospital
Sheffield
UK
利益声明
JTR is an author of several references cited in this topic.
Giovanni Barosi, MD
Director of the Laboratory of Clinical Epidemiology
IRCCS Policlinico S. Matteo Foundation
Pavia
Italy
利益声明
GB declares that he has no competing interests.
Richard Silver, MD
Myeloproliferative Disorders Program Specialist
Department of Medicine
Division of Hematology and Medical Oncology
Weill Cornell Medical College
New York
NY
利益声明
RS is an author of a reference cited in this topic.
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
Tefferi A. Primary myelofibrosis: 2023 update on diagnosis, risk-stratification, and management. Am J Hematol. 2023 May;98(5):801-21.全文 摘要
National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: myeloproliferative neoplasms [internet publication].全文
McLornan DP, Godfrey AL, Green A, et al. Diagnosis and evaluation of prognosis of myelofibrosis: a British Society for Haematology guideline. Br J Haematol. 2024 Jan;204(1):127-35.全文 摘要
Kröger N, Bacigalupo A, Barbui T, et al. Indication and management of allogeneic haematopoietic stem-cell transplantation in myelofibrosis: updated recommendations by the EBMT/ELN International Working Group. Lancet Haematol. 2024 Jan;11(1):e62-74. 摘要
McLornan DP, Psaila B, Ewing J, et al. The management of myelofibrosis: a British Society for Haematology guideline. Br J Haematol. 2024 Jan;204(1):136-50.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
- Polycythemia vera
- Essential thrombocythemia
- Chronic myeloid leukemia
更多 鉴别诊断指南
- NCCN clinical practice guidelines in oncology: myeloproliferative neoplasms
- NCCN clinical practice guidelines in oncology: hematopoietic cell transplantation (HCT)
更多 指南医学计算器
Dynamic International Prognostic Scoring System-Plus (DIPSS-Plus)
Mutation and Karyotype-Enhanced International Prognostic Scoring System for Primary Myelofibrosis in adults 70 and younger (MIPSS70+ version 2.0)
更多 医学计算器登录或订阅即可浏览 BMJ Best Practice 临床实践完整内容
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