Resumo
Definição
História e exame físico
Principais fatores diagnósticos
- abdominal fullness or discomfort
- splenomegaly
Outros fatores diagnósticos
- weakness and fatigue
- unexplained weight loss
- bruising and bleeding
- pallor
- recurrent infections
- hepatomegaly
- lymphadenopathy
- neurologic findings
- associated systemic immunologic disorders
Fatores de risco
- middle age
- male sex
- white ancestry
- western hemisphere location
- environmental exposures
- genetic predisposition
- Epstein-Barr virus
- infectious mononucleosis
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
Investigações a serem consideradas
- molecular analysis (for BRAF V600E mutation or IGHV4-34 rearrangement)
- CT chest, abdomen, and pelvis
Algoritmo de tratamento
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
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
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Declarações
As afiliações e declarações dos pareceristas referem--se ao momento da revisão.
Referências
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.

Diagnósticos diferenciais
- Chronic lymphocytic leukemia (CLL)
- Mantle cell lymphoma
- Prolymphocytic leukemia
Mais Diagnósticos diferenciaisGuidelines
- 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
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