Purpose@#
Acute promyelocytic leukemia (APL) is a
rare disease in
children and there are some different characteristics between
children and
adult . We aimed to evaluate
incidence , clinical characteristics and
treatment outcomes of pediatric APL in
Korea . @*Materials and
Methods @#Seventy-nine pediatric APL
patients diagnosed from January 2009 to December 2016 in 16 tertiary medical centers in
Korea were reviewed retrospectively. @*Results@#Of 801
acute myeloid leukemia children , 79 (9.9%) were diagnosed with APL. The median age at
diagnosis was 10.6 years (range, 1.3 to 18.0).
Male and
female ratio was 10.93. Thirty
patients (38.0%) had
white blood cell (WBC) count greater than 10×109/L at
diagnosis . All
patients received induction
therapy consisting of all-
trans retinoic acid and
chemotherapy . Five
patients (6.6%) died during
induction chemotherapy and 66
patients (86.8%) achieved complete remission (CR) after
induction chemotherapy . The
causes of death were three
intracranial hemorrhage , one
cerebral infarction , and one
sepsis . Five
patients (7.1%) suffered a
relapse during or after
maintenance chemotherapy . The estimated 4-year
event-free survival and overall
survival (OS) rates were 82.1%±4.4%, 89.7%±5.1%, respectively. The 4-year OS was significantly higher in
patients with initial WBC < 10×109/L than in those with initial WBC ≥ 10×109/L (p=0.020). @*Conclusion@#This study showed that the CR rates and
survival outcomes in Korean pediatric APL
patients were relatively good. The initial WBC count was the most important
prognostic factor and most
causes of death were related to serious
bleeding in the early stage of
treatment .