No abstract

Acute promyelocytic leukemia (APL) affects 10%−15% of children with acute myeloid leukemia. It is characterized molecularly by chromosomal translocation t(15;17) and the promyelocytic leukemia/retinoic acid receptor alpha (PML-RARA) fusion gene and protein resulting from the translocation. 1 The 3-year disease-free survival rate is 83.6% with all-trans retinoic acid (ATRA) and anthracycline treatment protocols. 2 The combination of ATRA and arsenic trioxide (ATO) is reportedly effective in pediatric APL and is expected to become the standard treatment. 3 However, ATO is associated with QT prolongation, a serious side effect.Congenital long QT syndrome (LQTS) is caused by a genetic abnormality of ion channels, which sometimes leads to torsades de pointes (TdP), causing syncope and sudden cardiac death. 4,5 Unlike congenital LQTS, acquired LQTS is caused by electrolyte abnormalities and many drugs, such as the antiemetic and antibacterial medications. 4 However, how anthracyclines might affect the QT interval, especially in children with congenital LQTS, remains elusive. This report describes the treatment of APL in a female with congenital LQTS using anthracyclines and ATRA, avoiding the use of ATO, and with frequent electrocardiogram (ECG) monitoring.