Resumo
Definition
History and exam
Key diagnostic factors
- presença de fatores de risco
- desconforto ou plenitude abdominal
- esplenomegalia
Other diagnostic factors
- fraqueza e fadiga
- perda de peso inexplicada
- hematomas e sangramento
- palidez
- infecções recorrentes
- hepatomegalia
- linfadenopatia
- achados neurológicos
- distúrbios imunológicos sistêmicos associados
Risk factors
- meia-idade
- sexo masculino
- ascendência branca
- localização no hemisfério ocidental
- exposições ambientais
- predisposição genética
- Vírus Epstein-Barr
- mononucleose infecciosa
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
Investigations to consider
- análise molecular (para mutação BRAF V600E ou rearranjo IGHV4-34)
- TC de tórax, abdome e pelve
Treatment algorithm
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
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
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.
Differentials
- Leucemia linfocítica crônica (LLC)
- Linfoma de células do manto
- Leucemia pró-linfocítica
More DifferentialsGuidelines
- 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)
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