Acute Promyelocytic Leukemia in Children Cancer Hospital Egypt

Abstract Background: Paediatric acute promyelocytic leukemia (APL) accounts for 5% to 15% of all myelocytic leukemia. A retrospective study of pediatric patients diagnosed and treated with APL was done from July 2012 till the end of December 2019 at CCHE, aimed to, report the prevalence, clinical features, outcomes, and risk factors causing induction failure and early deaths. Result: Sixty-two patients were reported, age above 10, initial unsatisfactory coagulation profile, and total leukocyte count above 30 103/mm affecting the 5 years (5y) overall (OS) and event-free survival (EFS), high promyelocyte count affecting the 5y EFS. Patients received protocol adopted from COG AAML1331 protocol. Prognostic factors causing early deaths during induction are high-risk patients with initial TLC >10x103/mm and initial promyelocytic count above 30% with significant P-value. In the female gender, wild FLT3 increases the occurrence of differentiation syndrome (DS). Receiving steroids with all-trans retinoic acid (ATRA) in induction may minimize the frequency of DS. Relapse affecting the outcome, Bone marrow transplantation was done for 4 patients of the relapsed patients, with 5y OS 37%, with no significance. In the current study, forty-five patients are alive in complete remission with 5y OS of 72.5%, with 5y EFS of 69.4%, respectively. Conclusion: pediatric APL outcome is affected by age above 10, initial unsatisfactory coagulation profile and promyelocyte count above 10%. Early death is affected by an initial leukocyte count above 10 x103/mm and, an initial promyelocytic count above 30%. Receiving steroids with ATRA may minimize the frequency of DS.