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Mielofibrose

  • Overview
  • Theory
  • Diagnosis
  • Management
  • Follow up
  • Resources
Última revisão: 16 Aug 2025
Última atualização: 16 Apr 2025

Resumo

Definição

História e exame físico

Principais fatores diagnósticos

  • presença de fatores de risco
  • sintomas de anemia (fadiga, fraqueza, dispneia, palpitações)
  • sintomas constitucionais (perda de peso, sudorese noturna, febre baixa, caquexia, fadiga e prurido)
  • esplenomegalia ± hepatomegalia
  • características da hematopoese extramedular
Detalhes completos

Outros fatores diagnósticos

  • características de hipertensão portal
  • dor nas articulações e nos ossos
  • perda auditiva
  • sangramento
  • infecções
Detalhes completos

Fatores de risco

  • exposição à radiação
  • exposição a solventes industriais
  • idade ≥65 anos
  • anormalidades citogenéticas
Detalhes completos

Investigações diagnósticas

Primeiras investigações a serem solicitadas

  • Hemograma completo com diferencial
  • esfregaço de sangue periférico
  • aspiração da medula óssea
  • biópsia da medula óssea
  • hibridização in situ fluorescente (FISH) ou reação em cadeia da polimerase via transcriptase reversa multiplex
  • análise de mutações genéticas
Detalhes completos

Investigações a serem consideradas

  • análise citogenética da medula óssea
  • ecocardiograma
  • ultrassonografia do local suspeito
  • cintigrafia com tecnécio-99
  • tomografia computadorizada (TC) do local suspeito
  • ressonância nuclear magnética (RNM) do local suspeito
  • ácido úrico sérico
  • fator antinuclear
  • título do fator reumatoide
  • níveis do complemento
  • teste de Coombs
Detalhes completos

Algoritmo de tratamento

AGUDA

menor risco: assintomático

risco baixo: sintomático

risco mais elevado: candidato mais jovem a transplante de células-tronco sem comorbidades

risco mais elevado: candidato a transplante de células-tronco >70 anos ou candidato a transplante de células-tronco mais jovem com comorbidades

risco mais elevado: não é candidato a transplante de células-tronco

Colaboradores

Autores

Jerry L. Spivak, MD
Jerry L. Spivak

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

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

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.

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.
  • Mielofibrose images

  • 鉴别诊断

    • Policitemia vera
    • Trombocitemia essencial
    • Leucemia mieloide crônica
    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+ versão 2.0)

    更多 医学计算器
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