How is high-risk acute promyelocytic leukemia (APL) treated?

Updated: May 03, 2019
  • Author: Sandy D Kotiah, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

In high-risk patients (WBC >10,000/μL), the NCCN recommends the following regimens for induction therapy [14] :

  • ATRA plus daunorubicin and cytarabine

  • ATRA plus age-adjusted idarubicin plus ATO

  • ATRA plus idarubicin 

For patients who are unable to tolerate anthracyclines, the NCCN recommends ATRA plus ATO.

Idarubicin has been shown to be slightly more effective than daunorubicin in younger patients with acute myelogenous leukemia (AML). The combination of ATRA with chemotherapy improves long-term survival and results in 85-90% complete remission rates.

The most effective and least toxic induction chemotherapy combination with ATRA has not been established. The PETHEMA (Programa de Estudio y Tratamiento de las Hemopatías Malignas) [24] and European APL 2000 [25] groups showed comparable and high complete remission rates with different induction chemotherapy regimens. The main difference was the addition of cytarabine by the APL 2000 group. The addition of cytarabine causes more myelosuppression, but the APL 2000 group reported a higher relapse risk when it was omitted. [25]


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