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
Treatment algorithm
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
Contributors
Authors
Jerry L. Spivak, MD

Professor of Medicine and Oncology
Division of Hematology
Johns Hopkins University School of Medicine
Baltimore
MD
Disclosures
JLS is an author of several references cited in this topic and has been reimbursed by GSK for a consultation.
Acknowledgements
Professor Jerry Spivak would like to gratefully acknowledge Dr Ashkan Emadi, a previous contributor to this topic.
Disclosures
AE declares that he has no competing interests.
Peer reviewers
John T. Reilly, BSc, MD, FRCP, FRCPATH
Professor and Consultant in Haematology
Royal Hallamshire Hospital
Sheffield
UK
Disclosures
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
Disclosures
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
Раскрытие информации
RS is an author of a reference cited in this topic.
Список литературы
Основные статьи
Tefferi A. Primary myelofibrosis: 2023 update on diagnosis, risk-stratification, and management. Am J Hematol. 2023 May;98(5):801-21.Полный текст Аннотация
National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: myeloproliferative neoplasms [internet publication].Полный текст
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.Полный текст Аннотация
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. Аннотация
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.Полный текст Аннотация
Статьи, указанные как источники
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
Больше Отличия指南
- NCCN clinical practice guidelines in oncology: myeloproliferative neoplasms
- NCCN clinical practice guidelines in oncology: hematopoietic cell transplantation (HCT)
Больше 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)
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