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Treatment of acute promyelocytic leukemia (APL), a subtype of acute myeloid leukemia (AML), differs from the usual AML treatment. This leukemia usually responds well to treatment, which is given in 3 phases:

• Induction
• Consolidation (also called intensification)
• Maintenance

Induction

Many children with APL have bleeding and blood-clotting issues when APL is diagnosed, which can cause serious problems during early treatment. Because of this, children with APL must be treated carefully and are often given an anticoagulant (“blood thinner”) to help prevent or treat these problems.

Children with APL get a non-chemotherapy drug similar to vitamin A called all-trans retinoic acid (ATRA). ATRA alone can often put APL into remission, but combining it with chemotherapy (usually daunorubicin and cytarabine) gives better long-term results. APL rarely spreads to the brain or spinal cord, so intrathecal chemotherapy is usually not needed.

In adults, ATRA is often combined with arsenic trioxide (ATO), another non-chemo drug, instead of chemo, as the initial treatment of APL. The results seem to be at least as good, and without some of the side effects of chemo. The combination of ATRA and ATO is now being studied in children as well.

Consolidation (intensification)

This is usually similar to induction, using both ATRA and chemotherapy (daunorubicin, sometimes along with cytarabine). Because of the success of this treatment, a stem cell transplant is not usually advised as long as the leukemia stays in remission.

ATRA plus ATO is also being studied as an option for consolidation therapy.

Maintenance

Children with APL may get maintenance therapy with ATRA (often with the chemo drugs methotrexate and 6-mercaptopurine) for about a year.

Relapsed APL

If the leukemia comes back after treatment, most often it can be put into a second remission. Arsenic trioxide is a drug that is very effective in this setting. ATRA plus chemo may be another option. A stem cell transplant may be considered once a second remission is achieved.