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Angeline Abraham
Aleena Sara Matthew
ETIOLOGY
GENETIC
Down syndrome
ENVIRONMENTAL
Fanconi anaemia
Ionizing radiations
Ataxia telengiectasia
Alkylating
agents(cyclophosphamide,carbo
platin)
Nitrosurea(nitrogen mustard)
Benzene
FAB CLASSIFICATION
PROGNOSTIC FEATURES
CLINICAL PRESENTATION
DIFFERENTIAL DIAGNOSIS
Infectious mononucleosis
Acute infectious lymphocytosis
Idiopathic thrombocytopenic purpura
Aplastic anemia
Juvenile rheumatoid arthirits
INVESTIGATIONS
Full blood count
Peripheral smear
Immunophenotyping
Differentiates the cellular lineages into pre-B, T cell and mature B cell.
CSF cytology
MANAGEMENT
Risk based approach
AGE
WBC COUNT
1-9 yrs
<50,000/mm3
Average risk
3 important determinants
are :
Infants <1yr
Children >10yr
Higher risk
Age at presentation
Infants <6m
Extremely poor
outcome
4 stages :
1. Induction therapy(to attain remission)
2. CNS prophylaxis or CNS preventive therapy
3. Intensification(consolidation)
4. Maintenance therapy(continuation)
Supportive care
Cotrimoxazole prophylaxis against Pneumocystis jiroveci
pneumonia
Hepatits B vaccine
Screening for HIV infection
PROGNOSIS
80% - longterm survivors in developed countries
20-30% - develop bone marrow relapse with current therapy