Info

🌱 來自: acute myelogenous leukemia (AML)

acute promyelocytic leukemia (APL)

(Blood 2009;113:1875)

Rare, ~8% of AML in U.S.

90% cure rates

• Atypical promyelocytes (large, granular cells; bilobed nuclei) in blood and bone marrow

• Defined by translocation of retinoic acid receptor: t(15;17); PML-RARA (>95% of cases)

APOLLO

• Medical emergency with DIC and bleeding common

• Remarkable responses to all-trans-retinoic acid (ATRA) & arsenic trioxide (ATO) which induce differentiation of leukemic blasts.

  • ∴ early initiation of ATRA if APL suspected

Non-high-risk APL 🍏

• ATRA + ATO (induction + 4 cycles consolidation)
• → CR ~100%;
• event-free survival 97% and overall survival 99% at 2 y (NEJM 2013;362:111)

High-risk APL 🍎

• WBC >10k at diagnosis.
• No clear consensus.
• In general, chemo (anthracycline or gemtuzumab ozogamicin) added to ATRA + ATO induction and consolidation.

Differentiation syndrome (ATRA)

References

atypical APL