Leucemia linfoblástica aguda en pacientes portadores de síndrome de Down

Abstract

Background: Children with Down's Syndrome (DS) present a higher incidence of Acute Lymphoblastic Leukemia (ALL) with more complications and shorter survival than healthy children. Objective: To describe clinical characteristics, laboratory and treatment results in patients with DS and ALL. Patients and Method: Retrospective analysis of 42 DS and ALL patients treated in three consecutive trials (1992,1996,2002) from the Pediatric National Cancer Program (PINDA). Clinical data, immunophenotype, cytogenetics and treatment results were analyzed. Results: There was no difference by age or gender, no patient presented LLA-T, t (9;22) o t (4;11). Of the 42 patients, 38 patients went into remission, 10 relapsed (26,3%), 11 died because of infection, none died from other toxicity. Survival at 5 years was 35 ± 9% (median of follow-up was 50 mo), similar for all protocols (p = 0,61). Conclusion: The group of patients with ALL and DS evaluated was not associated with classic treatment resistance factors. The relapse rate was not increased, if compared with non DS ALL patients; in this group the infections were the determinant factor for a lower survival. These patients can be treated with the current trials but they require a detailed infection care.