Summary
Definition
History and exam
Key diagnostic factors
- lymphadenopathy
- hepatosplenomegaly
- pallor, ecchymoses, or petechiae
- fever
- fatigue, dizziness, palpitations, and dyspnea
- bruising, epistaxis, menorrhagia
Other diagnostic factors
- focal neurologic signs, headache, papilledema, nuchal rigidity, and meningismus
- renal enlargement
- bone pain
- painless unilateral testicular enlargement
- abdominal pain
- mediastinal or abdominal mass
- pleural effusion
- skin infiltrations
Risk factors
- children less than 5 years of age
- genetic factors
- family history of ALL
- viruses
- environmental factors
- history of malignancy
- treatment with chemotherapy
- male sex
- Hispanic populations
- folate metabolism polymorphisms
- poor maternal diet
Diagnostic tests
1st tests to order
- CBC with differential
- peripheral blood smear
- serum electrolytes
- serum uric acid
- serum lactate dehydrogenase (LDH)
- renal function tests
- liver function tests
- coagulation profile
- bone marrow evaluation
- immunophenotyping
- cytogenetic analysis (karyotyping and fluorescence in situ hybridization [FISH])
- molecular studies (reverse transcriptase polymerase chain reaction [RT-PCR])
- next-generation sequencing (NGS) assay
- blood group and antibody screening
- antibody testing for infection
Tests to consider
- chest radiograph
- lumbar puncture
- pleural tap
- CT/MRI brain
- CT chest
- scrotal ultrasound
- baseline measurable residual disease (MRD) testing
- thiopurine methyltransferase (TPMT) phenotyping
- nudix hydrolase 15 (NUDT15) phenotyping
- HLA-typing
- echocardiogram or multigated acquisition (MUGA) scan
Treatment algorithm
adolescents and adults: newly diagnosed Ph+ B-ALL
adolescents and adults: newly diagnosed Ph-negative B-ALL or T-ALL
complete remission: standard-risk Ph-negative B-ALL or T-ALL
complete remission: Ph+ B-ALL and high-risk Ph-negative B-ALL or T-ALL
relapsed or refractory disease
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.
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