Outcome and prognosis of anaplastic large cell lymphoma in children: a report from the Taiwan Pediatric Oncology Group.

ABSTRACT

Studies of childhood anaplastic large cell lymphoma (ALCL) are less reported from East Asian countries. Clinical features and outcome of 90 children with ALCL in Taiwan were analyzed. The median age at diagnosis was 11.7 years. The most common presentation was lymph node involvement (86.7%). Advanced diseases accounted for 70% of patients at diagnosis. Most patients (93.1%) had positive staining for anaplastic lymphoma kinase. The five-year overall survival and event-free survival (EFS) rates were 79.7% and 73.3%, respectively. Bone marrow involvement, advanced stage, and thoracopulmonary ALCL were adverse prognostic factors for EFS (p=.05, .04, and .03, respectively). In multivariate analysis, only thoracopulmonary ALCL had a marginal significance on worse EFS (p= .054). We suggested that children with thoracopulmonary ALCL may need to intensify the treatment, and introduction of new targeted therapies for relapsed/refractory disease will be required.