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
- papilledema, nuchal rigidity, and meningismus
- focal neurologic signs
- painless unilateral testicular enlargement
- renal enlargement
- bone pain
- abdominal pain
- mediastinal or abdominal mass
- pleural effusion
- skin infiltrations
Risk factors
- children less than 5 years of age
- age in mid to late 30s
- age in mid 80s
- genetic factors
- family history of ALL
- viruses
- environmental factors
- history of malignancy
- treatment with chemotherapy
- male sex
- white population
- poor maternal diet
Diagnostic investigations
1st investigations to order
- CBC with differential
- serum electrolytes
- peripheral blood smear
- renal function
- liver function
- lactic dehydrogenase
- coagulation profile
- bone marrow aspiration and trephine biopsy
- immunophenotyping (on bone marrow, or peripheral blood if cell count is raised)
- thiopurine methyltransferase (TPMT) phenotyping
- nudix hydrolase 15 (NUDT15) phenotyping
- cytogenetics
- molecular studies
Investigations to consider
- HLA-typing
- chest x-ray
- lumbar puncture (LP)
- pleural tap
- CT/MRI brain
- CT
- minimal residual disease molecular samples
Treatment algorithm
newly diagnosed ALL without CNS disease
newly diagnosed ALL with CNS disease
complete remission: standard risk
complete remission: high risk
relapsed or refractory disease
Contributors
Authors
Melissa Ooi, MB BCh, BAO, MRCP(I), FRCPath, PhD
Consultant
Department of Haematology-Oncology
National University Cancer Institute
Singapore
Disclosures
MO declares that she has no competing interests.
Michelle Poon, MBBS, MRCP, FRCPath
Senior Consultant
Department of Haematology-Oncology
National University Cancer Institute
Singapore
Disclosures
None declared.
Esther Chan, MD
Consultant
Department of Haematology-Oncology
National University Cancer Institute
Singapore
Disclosures
EC declares that she has no competing interests.
Chin Hin Ng, MD, MRCP, FRCPath
Consultant
Department of Haematology-Oncology
National University Cancer Institute
Singapore
Disclosures
CHN declares that he has no competing interests.
Acknowledgements
Dr Melissa Ooi, Dr Michelle Poon, Dr Esther Chan, and Dr Chin Hin Ng would like to gratefully acknowledge Dr Arati V. Rao, Dr Matthew Smith, Dr Samer Bleibel, and Dr Robert Leonard, previous contributors to this topic.
Disclosures
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.
Shankaranarayanan 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.
Differentials
- Acute myeloid leukemias (AML)
- Reactive lymphocytosis ("leukemoid reaction")
- Small-cell lung cancer
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