When viewing this topic in a different language, you may notice some differences in the way the content is structured, but it still reflects the latest evidence-based guidance.

Leucemia de células pilosas

Última revisão: 16 Aug 2025
Última atualização: 25 Oct 2024

Resumo

Definition

History and exam

Key diagnostic factors

  • presença de fatores de risco
  • desconforto ou plenitude abdominal
  • esplenomegalia
Full details

Other diagnostic factors

  • fraqueza e fadiga
  • perda de peso inexplicada
  • hematomas e sangramento
  • palidez
  • infecções recorrentes
  • hepatomegalia
  • linfadenopatia
  • achados neurológicos
  • distúrbios imunológicos sistêmicos associados
Full details

Risk factors

  • meia-idade
  • sexo masculino
  • ascendência branca
  • localização no hemisfério ocidental
  • exposições ambientais
  • predisposição genética
  • Vírus Epstein-Barr
  • mononucleose infecciosa
Full details

Diagnostic investigations

1st investigations to order

  • Hemograma completo com diferencial
  • esfregaço de sangue periférico
  • biópsia e aspiração da medula óssea por trefina (avaliação morfológica)
  • imunofenotipagem (imuno-histoquímica ou citometria de fluxo)
  • perfil metabólico completo
  • lactato desidrogenase (LDH) sérica
  • sorologia viral para hepatite B e C
Full details

Investigations to consider

  • análise molecular (para mutação BRAF V600E ou rearranjo IGHV4-34)
  • TC de tórax, abdome e pelve
Full details

Treatment algorithm

ACUTE

nenhuma indicação(ões) para tratamento

indicação(ões) para tratamento presente(s): ausência de ruptura esplênica ou esplenomegalia maciça ou trombocitopenia acentuada que impeça a quimioterapia

indicação(ões) para tratamento presente(s): com esplenomegalia sintomática maciça ou ruptura esplênica ou trombocitopenia acentuada que impeça a quimioterapia

ONGOING

recidiva precoce (<2 anos) ou doença refratária

recidiva tardia (≥2 anos)

Contributors

Authors

Ambuj Kumar, MD, MPH

Professor

USF Health Office of Research

Department of Internal Medicine, College of Medicine

Moffitt Cancer Center & Research Institute, Department of Health Outcomes & Behavior

University of South Florida

Tampa

FL

Disclosures

AK declares that he has no competing interests.

Mohamed A. Kharfan-Dabaja, MD, MBA, FACP

Professor

Division of Hematology-Oncology

Blood and Marrow Transplantation Program

Mayo Clinic

Jacksonville

FL

Disclosures

MKD declares that he has no competing interests.

Acknowledgements

Dr Ambuj Kumar and Dr Mohamed Kharfan-Dabaja would like to gratefully acknowledge Dr Benjamin Djulbegovic, a previous contributor to this topic.

Disclosures

BD declares that he has no competing interests.

Peer reviewers

Daniel Catovsky, MD, FRCP, FRCPath, DSc, FMedSc

Consultant Haemato-Oncologist

Section of Haemato-Oncology

Brookes Lawley Institute of Cancer

Sutton

UK

Disclosures

DC declares that he has no competing interests.

Roger Lyons, MD

Clinical Professor of Medicine

University of Texas Health Science Center San Antonio

San Antonio

TX

Disclosures

RL declares that he has no competing interests.

Rebecca Connor, MD

Chief Fellow

Section of Hematology and Oncology

Department of Internal Medicine

Wake Forest University Baptist Medical Center

Winston-Salem

NC

Disclosures

RC declares that she has no competing interests.

Claire Dearden, BSc, MD, FRCP, FRCPath

Consultant Haematologist

The Royal Marsden Hospital

Sutton

Surrey

UK

Disclosures

CD 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

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.

Key articles

Parry-Jones N, Joshi A, Forconi F, et al. Guideline for diagnosis and management of hairy cell leukaemia (HCL) and hairy cell variant (HCL-V). Br J Haematol. 2020 Dec;191(5):730-7.Full text  Abstract

Troussard X, Maître E, Paillassa J. Hairy cell leukemia 2024: update on diagnosis, risk-stratification, and treatment - annual updates in hematological malignancies. Am J Hematol. 2024 Apr;99(4):679-96.Full text  Abstract

Grever MR, Abdel-Wahab O, Andritsos LA, et al. Consensus guidelines for the diagnosis and management of patients with classic hairy cell leukemia. Blood. 2017 Feb 2;129(5):553-60.Full text  Abstract

National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: hairy cell leukemia [internet publication].Full text

Robak T, Matutes E, Catovsky D, et al. Hairy cell leukaemia: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2015 Sep;26(suppl 5):v100-7.Full text  Abstract

Troussard X, Maître E, Cornet E. Hairy cell leukemia 2022: update on diagnosis, risk-stratification, and treatment. Am J Hematol. 2022 Feb 1;97(2):226-36.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.
  • Leucemia de células pilosas images
  • Differentials

    • Leucemia linfocítica crônica (LLC)
    • Linfoma de células do manto
    • Leucemia pró-linfocítica
    More Differentials
  • Guidelines

    • NCCN clinical practice guidelines in oncology: hairy cell leukemia
    • Guideline for diagnosis and management of hairy cell leukaemia (HCL) and hairy cell variant (HCL-V)
    More Guidelines
  • padlock-lockedLog in or subscribe to access all of BMJ Best Practice

Use of this content is subject to our disclaimer