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Hairy cell leukemia

Evidencia revisada por última vez: 16 Jul 2026
Tema actualizado por última vez: 07 Aug 2026

Resumen

Definición

Anamnesis y examen

Principales factores de diagnóstico

  • abdominal fullness or discomfort
  • splenomegaly
Todos los datos

Otros factores de diagnóstico

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

Factores de riesgo

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

Pruebas diagnósticas

Primeras pruebas diagnósticas para solicitar

  • 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
Todos los datos

Pruebas diagnósticas que deben considerarse

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

Algoritmo de tratamiento

Agudo

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

En curso

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

Divulgaciones

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

Divulgaciones

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

Agradecimientos

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

Divulgaciones

BD declares that he has no competing interests.

Revisores por pares

Roger Lyons, MD

Clinical Professor of Medicine

University of Texas Health Science Center San Antonio

San Antonio, TX

Divulgaciones

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

Divulgaciones

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

Divulgaciones

CD declares that she has no competing interests.

Agradecimiento de los revisores por pares

Los temas de BMJ Best Practice se actualizan de forma continua de acuerdo con los desarrollos en la evidencia y en las guías. Los revisores por pares listados aquí han revisado el contenido al menos una vez durante la historia del tema.

Divulgaciones

Las afiliaciones y divulgaciones de los revisores por pares se refieren al momento de la revisión.

Referencias

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Artículos principales

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  Resumen

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  Resumen

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  Resumen

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  Resumen

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  Resumen

Artículos de referencia

Una lista completa de las fuentes a las que se hace referencia en este tema está disponible para los usuarios con acceso a todo BMJ Best Practice.
  • Hairy cell leukemia images
  • Diferenciales

    • Chronic lymphocytic leukemia (CLL)
    • Mantle cell lymphoma
    • Prolymphocytic leukemia
    Más Diferenciales
  • Guías de práctica clínica

    • 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
    Más Guías de práctica clínica
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