A Clinical Study For Childhood With Acute Promyelocytic Leukemia

Objective To evaluate the clinical features, cytogenetics and outcome of acute promyelocytic leukaemia(APL) in childhood. Methods Since November 1991,102 children (younger than 18 years) proven APL were included. Fourteen patients failed to be treated due to the economical reason. Data from others were analyzed with SPSS 10.0 system software. Result Sixty-one boys (59.8%) and 41 girls (40.2%) were included in this study. The median age at diagnosis was 13(3-18) years old. Childhood APL were characterized by anemia, hemorrhage, and fever. The WBC count at presentation was more than 10×10~9/L in 32 patients (31.4%). The immunophenotype of APL was characterized by the high express CD9,CD13,CD33,CD64,CD38,CD117,HIT4 (CD99R) and the low express CD34,HLA-DR. Cytogenetic studies demonstrated the characteristic 15; 17 translocation in about 64.3% of the 56 patients analyzed, PML/RARαpositive cases was 93.8% of the 64 patients analyzed. Additional cytogenetic changes did not influence the outcome of patients with newly diagnosed acute promyelocytie leukemia treated with an ATRA plus anthracyline- based protocol. Seventy-five children (85.2%) achieved complete remission (CR). Early death from hemorrhage occurred in eleven patients. Since July 2003,six patients received the consolidation therapy with anthracycline monochemotherapy after complete remission. Hematologic toxicity of them was smaller than those who were treated with combined chemotherapy (anthracycline and cytosine arabinoside). Six clinical relapse and two molecular relapse were also observed in the bone marrow of 88 patients. The 5-year cumulative incidence of relapse was 16%, whereas event-flee, disease-free and overall survival rates were 72.3%, 85.0% and 85.8%, respectively. The WBC count at presentation may be the most important risk factor of the outcome. Conclusion Treated with ATRA combined with chemotherapy for induce and maintenance therapy provided favorable results in children with APL. To reduce the rate of early death and relapse was the major subject for the progressive of the outcome.