Summary
Definition
History and exam
Key diagnostic factors
- presença de fatores de risco
- linfadenopatia
- hepatoesplenomegalia
- palidez, equimoses ou petéquias
- sintomas constitucionais (febres, sudorese noturna, perda de peso)
- infecções recorrentes
- fadiga, tontura, palpitações e dispneia
- hematomas, epistaxe, sangramento menstrual intenso
Other diagnostic factors
- alterações do estado mental, sinais/deficits neurológicos focais, cefaleia, papiledema, rigidez da nuca e meningismo
- aumento renal
- dor óssea
- aumento testicular unilateral e indolor
- dor abdominal
- massa mediastinal ou abdominal
- derrame pleural
- infiltrações na pele
Risk factors
- crianças <5 anos de idade
- fatores genéticos
- história familiar de LLA
- infecções virais
- fatores ambientais
- história de malignidade
- tratamento com quimioterapia
- sexo masculino
- Populações hispânicas
- polimorfismos do metabolismo do folato
- dieta materna inadequada
Diagnostic tests
1st tests to order
- Hemograma completo com diferencial
- esfregaço de sangue periférico
- eletrólitos séricos
- ácido úrico sérico
- lactato desidrogenase (LDH) sérica
- testes de função renal
- testes da função hepática
- perfil de coagulação
- avaliação da medula óssea (citomorfologia)
- imunofenotipagem
- análise citogenética (cariotipagem e hibridização in situ fluorescente [FISH])
- testes moleculares (reação em cadeia da polimerase via transcriptase reversa [RT-PCR])
- ensaio de sequenciamento de nova geração
- grupo sanguíneo e rastreamento de anticorpos
- testagem de anticorpos para infecção
Tests to consider
- punção lombar
- radiografia torácica
- toracocentese
- TC/RNM do crânio
- TC do pescoço, tórax, abdome, pelve
- ultrassonografia escrotal
- teste de doença residual mensurável (DRM) basal
- fenotipagem da tiopurina metiltransferase (TPMT)
- fenotipagem da nudix hidrolase 15 (NUDT15)
- tipagem do antígeno leucocitário humano (HLA)
- ecocardiograma ou angiografia sincronizada multinuclear (MUGA)
Treatment algorithm
adolescentes e adultos: LLA-B Ph+ recém-diagnosticada
adolescentes e adultos: LLA-B negativa para Ph ou LLA-T recém-diagnosticada
remissão completa: LLA-B positiva para Ph
remissão completa: LLA-B negativa para Ph
remissão completa: LLA-T
doença recidivante ou refratária
Contributors
Authors
Ryan D. Cassaday, MD
Associate Professor
Division of Hematology and Oncology, University of Washington School of Medicine
Clinical Research Division, Fred Hutchinson Cancer Center
Seattle
WA
Disclosures
RDC has received research funding from Amgen, Jazz Pharmaceuticals, Incyte, Kite/Gilead, Merck, Pfizer, Servier, and Vanda Pharmaceuticals; honoraria/consulting from Amgen, Jazz, Kite/Gilead, and Pfizer; serves on a board/advisory committee for Autolus and PeproMene Bio; and his spouse has been employed by and owned stock in Seagen. RDC is an author of several articles cited in the topic.
Acknowledgements
Dr Ryan D. Cassaday would like to gratefully acknowledge Dr Melissa Ooi, Dr Michelle Poon, Dr Esther Chan, Dr Chin Hin Ng, Dr Arati V. Rao, Dr Matthew Smith, Dr Samer Bleibel, and Dr Robert Leonard, previous contributors to this topic.
Disclosures
MO, MP, EC, CHN, AVR, MS, SB, and RL declare that they have no competing interests.
Peer reviewers
Olga Kozyreva, MD
Staff Physician
Department of Hematology and Oncology
New England Medical Center
Tufts University
Boston
MA
Disclosures
OK declares that she has no competing interests.
Shankaranarayana Paneesha, MD, MRCP, FRCPath
Consultant Haematologist
Department of Haematology and Stem Cell Transplantation
Heartlands Hospital
Birmingham
UK
Disclosures
SP declares that he has no competing interests.
References
Key articles
Alaggio R, Amador C, Anagnostopoulos I, et al. The 5th edition of the World Health Organization classification of haematolymphoid tumours: lymphoid neoplasms. Leukemia. 2022 Jul;36(7):1720-48.Full text Abstract
Arber DA, Orazi A, Hasserjian RP, et al. International consensus classification of myeloid neoplasms and acute leukemias: integrating morphologic, clinical, and genomic data. Blood. 2022 Sep 15;140(11):1200-8.Full text Abstract
National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: acute lymphoblastic leukemia [internet publication].Full text
Hoelzer D, Bassan R, Dombret H, et al. Acute lymphoblastic leukaemia in adult patients: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2016 Sep;27(suppl 5):v69-82.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.
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