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 tests
1st tests 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
Tests 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
利益声明
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
利益声明
MKD declares that he has no competing interests.
鸣谢
Dr Ambuj Kumar and Dr Mohamed Kharfan-Dabaja would like to gratefully acknowledge Dr Benjamin Djulbegovic, a previous contributor to this topic.
利益声明
BD declares that he has no competing interests.
同行评议者
Daniel Catovsky, MD, FRCP, FRCPath, DSc, FMedSc
Consultant Haemato-Oncologist
Section of Haemato-Oncology
Brookes Lawley Institute of Cancer
Sutton
UK
利益声明
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
利益声明
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
利益声明
RC declares that she has no competing interests.
Claire Dearden, BSc, MD, FRCP, FRCPath
Consultant Haematologist
The Royal Marsden Hospital
Sutton
Surrey
UK
利益声明
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.
参考文献
关键文献
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.全文 摘要
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.全文 摘要
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.全文 摘要
National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: hairy cell leukemia [internet publication].全文
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.全文 摘要
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.全文 摘要
参考文献
A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
鉴别诊断
- Leucemia linfocítica crônica (LLC)
- Linfoma de células do manto
- Leucemia pró-linfocítica
更多 鉴别诊断指南
- 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)
更多 指南
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