Resumen
Definición
Anamnesis y examen
Principales factores de diagnóstico
- abdominal fullness or discomfort
- splenomegaly
Otros factores de diagnóstico
- weakness and fatigue
- unexplained weight loss
- bruising and bleeding
- pallor
- recurrent infections
- hepatomegaly
- lymphadenopathy
- neurologic findings
- associated systemic immunologic disorders
Factores de riesgo
- middle age
- male sex
- white ancestry
- western hemisphere location
- environmental exposures
- genetic predisposition
- Epstein-Barr virus
- infectious mononucleosis
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
Pruebas diagnósticas que deben considerarse
- molecular analysis (for BRAF V600E mutation or IGHV4-34 rearrangement)
- CT chest, abdomen, and pelvis
Algoritmo de tratamiento
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
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 no competing interests.
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
Daniel Catovsky, MD, FRCP, FRCPath, DSc, FMedSc
Consultant Haemato-Oncologist
Section of Haemato-Oncology
Brookes Lawley Institute of Cancer
Sutton
UK
Divulgaciones
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
Divulgaciones
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
Divulgaciones
RC declares that she has no competing interests.
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
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
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.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
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.

Diferenciales
- Chronic lymphocytic leukemia (CLL)
- Mantle cell lymphoma
- Prolymphocytic leukemia
Más DiferencialesGuías de práctica clínica
- 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)
Más Guías de práctica clínicaInicie sesión o suscríbase para acceder a todo el BMJ Best Practice
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