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Hairy cell leukemia

Última revisão das evidências: 9 Aug 2026
Última atualização do tópico: 07 Aug 2026

Resumo

Definição

História e exame físico

Principais fatores diagnósticos

  • abdominal fullness or discomfort
  • splenomegaly
Detalhes completos

Outros fatores diagnósticos

  • weakness and fatigue
  • unexplained weight loss
  • bruising and bleeding
  • pallor
  • recurrent infections
  • hepatomegaly
  • lymphadenopathy
  • neurologic findings
  • associated systemic immunologic disorders
Detalhes completos

Fatores de risco

  • middle age
  • male sex
  • white ancestry
  • western hemisphere location
  • environmental exposures
  • genetic predisposition
  • Epstein-Barr virus
  • infectious mononucleosis
Detalhes completos

Investigações diagnósticas

Primeiras investigações a serem solicitadas

  • CBC with differential
  • peripheral blood smear
  • bone marrow trephine biopsy and aspiration (morphology assessment)
  • immunophenotyping (immunohistochemistry or flow cytometry)
  • comprehensive metabolic panel
  • serum lactate dehydrogenase (LDH)
  • viral serology for hepatitis B and C
Detalhes completos

Investigações a serem consideradas

  • molecular analysis (for BRAF V600E mutation or IGHV4-34 rearrangement)
  • CT chest, abdomen, and pelvis
Detalhes completos

Algoritmo de tratamento

AGUDA

no indication(s) for treatment

indication(s) for treatment present: without splenic rupture or massive splenomegaly or marked thrombocytopenia precluding chemotherapy

indication(s) for treatment present: with massive symptomatic splenomegaly or splenic rupture or marked thrombocytopenia precluding chemotherapy

CONTÍNUA

early relapse (<2 years) or refractory disease

late relapse (≥2 years)

Colaboradores

Autores

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

Declarações

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

Declarações

MKD declares that he has attended an advisory board for Incyte Corporation.

Agradecimentos

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

Declarações

BD declares that he has no competing interests.

Revisores

Roger Lyons, MD

Clinical Professor of Medicine

University of Texas Health Science Center San Antonio

San Antonio, TX

Declarações

RL declares that he has no competing interests.

Christopher D'Angelo, MD

Assistant Professor

Division of Oncology & Hematology

University of Nebraska Medical Center

Omaha, NE

Declarações

CDA declares that he has received consulting fees from Abbvie, Beigene, Genmab, Curis Inc, Bristol Myers Squibb.

Claire Dearden, BSc, MD, FRCP, FRCPath

Consultant Haematologist

The Royal Marsden Hospital

Sutton

Surrey

UK

Declarações

CD declares that she has no competing interests.

Créditos aos pareceristas

Os tópicos do BMJ Best Practice são constantemente atualizados, seguindo os desenvolvimentos das evidências e das diretrizes. Os pareceristas aqui listados revisaram o conteúdo pelo menos uma vez durante a história do tópico.

Declarações

As afiliações e declarações dos pareceristas referem--se ao momento da revisão.

Referências

Nossas equipes internas de editoria e de evidências trabalham em conjunto com colaboradores internacionais especializados e pares revisores para garantir que forneçamos acesso às informações o mais clinicamente relevantes possível.

Principais artigos

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.Texto completo  Resumo

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.Texto completo  Resumo

Zent CS, Tiacci E, Kreitman RJ, et al. Updated consensus guidelines for the diagnosis and management of patients with HCL and HCL variant. Blood. 2026 Jul 2;148(1):31-42.Texto completo  Resumo

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

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.Texto completo  Resumo

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.Texto completo  Resumo

Artigos de referência

Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.
  • Hairy cell leukemia images
  • Diagnósticos diferenciais

    • Chronic lymphocytic leukemia (CLL)
    • Mantle cell lymphoma
    • Prolymphocytic leukemia
    Mais Diagnósticos diferenciais
  • Guidelines

    • Updated consensus guidelines for the diagnosis and management of patients with hairy cell leukemia (HCL) and HCL-variant
    • NCCN clinical practice guidelines in oncology: hairy cell leukemia
    Mais Guidelines
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