Pediatric Acute Lymphocytic Leukemia
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Introduction
The Case of...
a simple case introducing clincial presentation and calling for a differential diagnosis to get students thinking.
Causes and Risk Factors
largely unknown
- existing myelodysplasia
- prior chemotherapy
- t 4:11 translocation in the MLL gene
- Down syndrome
- radiation
Pathophysiology
- M0: undifferentiated
- M1: no maturation
- M2: granulocytic maturation
- M3: promyelocytic
- M4: myelomonocytic
- M5: monocytic
- M6: erythroleukemic
- M7: megakaryocytic
M5 is most common in children. Bulky gum disease is a feature.
M3 is relatively common. Bleeding tendency accompanies.
APL is another form with better prognosis; use all-trans retinoic acid and arsenic trioixide to treat.
Signs and Symptoms
- history
- physical exam
History
more systemic symptoms than ALL
- pallor and tiredness from anemia
- recurrent infections
- bruising and bleeding from thrombocytopenia
- gum disease
- pre-existing myelodysplasia
- lymphadenopathy
- hepatosplenomegaly
- testicular enlargement
- headaches and cranial nerve palsies
- skin rashes
- occasional mediastinal masses
- bone pain and fractures
- gum hypertrophy
Physical Exam
Investigations
- lab investigations
- diagnostic imaging
Lab Investigations
CBC
- may be normal or mildly abnormal
- usually pancytopenia
- blasts in peripheral blood
- occasional hyperleukocytosis
bone marrow
- biopsy: histology and immunocytochemistry
- smear: morphology, flow cytometry, cytogenetics, molecular genetics
lumbar puncture
lymph node biopsy
mass biopsy
Diagnostic Imaging
Differential Diagnosis
Treatments
chemotherapy
- induction: 5 intensive blocks over 6 months
- intense - mandatory hospitalization and high risk of severe infection
stem cell /bone marrow transplants an early option.
supportive care:
- infection prophylaxis and treatment
- blood and platelet transfusions
- organ-specific support
- nutrition
- psychosocial care
Consequences and Course
overall survival is about 65%
relapses are less treatable.
Resources and References
Topic Development
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