Acute promyelocytic leukemia (APL) is a distinct subtype of acute myel
oblastic leukemia (AML). In this report, we present the clinical featu
res, management, and outcome of pediatric patients with APL treated wi
th all-trans-retinoic acid (ATRA). Of 52 newly diagnosed cases of APL
between February 1992 and December 1996, 15 were in the pediatric age
group (younger than 15 years). Four patients were treated with ATRA al
one and II were allocated to receive ATRA followed by chemotherapy. Ei
ghty-six percent of the patients achieved a complete response. The pat
ients who received ATRA alone as maintenance therapy had relapses with
a median duration of remission of 8 months (range 6-12). The patients
who received ATRA, followed by consolidation chemotherapy, had a prol
onged duration of remission, with a median of 20 months (range 13-28).
In addition, rapid correction of coagulopathy was observed in these p
atients. The median duration for correction of coagulopathy was 7 days
(range 5-11) and the median duration for recovery from neutropenia af
ter chemotherapy was 10 daps (range 7-20). Two major side effects of A
TRA were hyperleukocytosis and retinoic acid syndrome. Significantly p
rolonged disease-free survival was seen in patients who received ATRA
with chemotherapy. APL is not uncommon in the pediatric age group. ATR
A was well-tolerated by these patients. Consolidation with chemotherap
y helps in prolonging the disease-free survival in patients with APL i
n comparison to treatment with ATRA alone.