Treatment of acute promyelocytic leukemia (APL), the M3 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:
- Consolidation (also called intensification)
Many children with APL have bleeding and blood-clotting issues at the time 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.
Along with the possible side effects from the chemotherapy drugs, ATRA can cause a problem called differentiation syndrome (formerly known as retinoic acid syndrome). This can include breathing problems from fluid buildup in the lungs, low blood pressure, kidney damage, and severe fluid buildup elsewhere in the body. It can often be treated by stopping the ATRA for a while and giving a steroid such as dexamethasone.
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.
Children with APL may get maintenance therapy with ATRA (often with the chemo drugs methotrexate and 6-mercaptopurine) for about a year.
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, although it can sometimes cause problems with heart rhythms. Children getting this drug need to have their blood mineral levels watched closely. ATRA plus chemotherapy may be another option. A stem cell transplant may be considered once a second remission is achieved.
Last Revised: 02/03/2016