Clinical Manifestations
Differential Diagnosis
- Primary: suspect when multiple lineages affected, significant leukocytosis, lymphadenopathy, splenomegaly
- Secondary
- Acute infection (30-50%)
- Chronic inflammation
- Stress (MI, surgery, burn) (30-50%)
- Steroids
- Pregnancy
- Cigarette smoking
Investigations
- Urgent blood film review to r/o APL
Management
Acute
- Rule out DIC (especially seen in APL)
- More common in AML than any other
- Lungs and brain most commonly affected
- Cytoreduction
- Induction chemotherapy as soon as possilble
- Overnight, give hydroxyurea 2g q6h
- Can develop tumour lysis syndrome
- No evidence for leukopheresis
Suspected APL
- Consult Hematology overnight
- Start ATRA 45mg/m2 divided BID
- Beware differentiation syndrome, with fever, effusion, dyspnea, hypotension; treated with steroids
- Beware DIC
Chronic
AML
- Induction with 3+7: 3 days of daunorubicin and 7 days of cytarabine, includes about a month as inpatient
- BM to confirm induction, then consolidation
- Low risk: 2-3 more cycles of same
- High/poor risk: 1-2 more cycles, then alloSCT
APL
ALL
- Dana-Farber protocol
- 2+ years of chemo + steroids
- For Philadelphia positive disease, add imatinib