Clinical Analysis Of Childhood APL With APL2008

Objective To evaluate the treatment of current status and prognosisin childhood APL with APL2008, which was administrated since2008byour centre.Methods43children with newly diagnosed APL between2008to2014were studied retrospectively. Treatment options and current statuswere summarized from28patients who received APL2008therapy.Result Studied43patients were at median age of8years and4months, with28boys and15girls. The main clinical manifestations wereinfection, anemia, bleeding, fever, hepatomegaly, splenomegaly andlymphadenopathy. The proportions of low, intermediate and high-riskgroups were27.9%、48.8%and23.3%, respectively.11cases could bediagnosed as DIC. Bone marrow morphology showed abnormal elevationof promyelocyte.37patients had distinctive immunophenotype such asfrequent expression of CD33, CD117and MPO. PML/RARα fusion genepositive rate was100%in43children and cytogenetic analysis werepositive in37cases, of which specific genetic lesion in APL cells with t(15;17)(q22;q12) was found in28cases, and karyotypes was found in9cases as infrequent chromosomal abnormalities. In43patients,4caseswere early dead from intracranial hemorrhage at early stage, and11caseswere given up early. There were only2cases dead,2cases relapsed and1case lost among28APL children, which enabled efficacy analysis.96.4%of these28cases achieved HCR. The2-year Kaplan-Meier estimates of OSand EFS were85.9%±7.6%and80.4%±8.8%. But OS and EFS would be94.7%±5.1%and88.9%±7.4%if3patients who had non-standardtreatment were excluded.Conclusions Childhood APL were characterized by anemia,bleeding, fever and infiltration. APL’s coincidence rate betweenPML/RARa fusion gene and morphology, immunology and cytogeneticswere95.3%、90.2%and87.8%, respectively. APL2008significantlyimproved the prognosis of APL.