Summary
Definition
A LCP é uma neoplasia de células B maduras indolente. É considerada um tipo de linfoma não Hodgkin. Observadas ao microscópio, as células leucêmicas apresentam delicadas projeções citoplasmáticas que lembram pelos (“células pilosas”). A LCP tem uma apresentação característica de pancitopenia, esplenomegalia e células pilosas no sangue periférico, medula óssea e outros órgãos hematopoiéticos.[Figure caption and citation for the preceding image starts]: Citospina preparada a partir do aspirado de medula óssea ilustrando a citologia celular típica, com núcleos ovais e em forma de feijão e quantidades moderadas de citoplasma com bordas citoplasmáticas irregulares (Wright Giemsa, óleo 100x)Do acervo de Lynn Moscinski, MD [Citation ends].
[Figure caption and citation for the preceding image starts]: Seções da punção por agulha grossa (core biopsy) demonstrando linfócitos com citoplasma óbvio no interstício medular, associados à dilatação dos seios medulares e a coleções de eritrócitos (H&E, óleo 50x)Do acervo de Lynn Moscinski, MD [Citation ends].
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 attended an advisory board for Incyte Corporation.
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
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.
Christopher D'Angelo, MD
Assistant Professor
Division of Oncology & Hematology
University of Nebraska Medical Center
Omaha, NE
Disclosures
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
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
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.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
- 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
More Guidelines
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