ACUTE PROMYELOCYTIC LEUKEMIA IN CHILDREN: A SINGLE CENTRE EXPERIENCE FROM TURKEY
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Keywords
Acute promyelocytic leukemia, Hypogranular variant APL, ATRA toxicity
Abstract
Background and objectives: Acute promyelocytic leukemia (APL), characterized by tendency to hemorrhage and excellent response to all-trans retinoic acid (ATRA), is a distinct subtype of acute myeloid leukemia (AML). In this retrospective study, we aimed to determine the incidence, clinical symptoms, toxicities and outcome of children with APL in our center. Methods: We retrospectively reviewed the medical records of children (age < 18 years) diagnosed with APL at our pediatric hematology department between January 2006-December 2016.
Results: Pediatric APL represents 20.5% of AML cases in this cohort. Most of the cases presented as classical M3, albeit hypogranular variant was described in 12% of the cohort. Patients with hypogranular variant APL were differed from classical APL by co-expression of CD2 and CD34. About ¾ of APL patients had hemorrhagic findings at admission or at initial phase of the treatment. Severe bleeding manifested as intracranial hemorrhage was present in three patients and intracranial arterial thrombosis was present in one. Five patients showed side effects of ATRA such as pseudotumor cerebri, dilated cardiomyopathy, and pulmonary infiltrates. Six year overall survival (OS) and early death rate was found to be 82.5% and 12% respectively.
Conclusions: A high frequency (20.5%) of APL was noted among children with AML in this single center study. The overall mortality rate was 17.5%. Since the induction death rate was 12% and life threatening bleeding was the major problem, awareness and urgent treatment are critical factors to reduce early losses.
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