title: [Acute leukemia in patients with Down syndrome].
abstract: BACKGROUND
  Children with Down syndrome (DS) have a higher risk of acute leukemia than the remaining pediatric population. A favorable outcome of acute myeloid leukemia (AML) has recently been described in these patients whereas the prognosis of acute lymphoblastic leukemias (ALL) is similar to that in other children. The main cause of morbidity and mortality in children with Down syndrome are complications related to chemotherapy, leading to numerous modifications in treatment protocols.
  
  
  OBJECTIVE
  To characterize acute leukemias in children with Down syndrome in our center and determine their clinical outcome.
  
  
  RESULTS
  Between 1990 and 2002, 214 children were diagnosed with acute leukemia at the Niño Jesus Hospital (40 with AML and 174 with ALL). Of these, eight children (3.8 %) had Down syndrome. AML (2/40) represented 5 % of myeloid leukemias and ALL (6/174) represented 3.4 % of lymphoblastic leukemias. The most frequent complication was hematologic toxicity due to chemotherapy, causing a high incidence of infections: pneumonia (5/8) and bacteriemia (5/8). In all patients, these complications led to treatment interruption or dose reduction. Two children died from treatment-related toxicity. Of these, one with AML developed fulminant sepsis due to Candida infection and the other, diagnosed with high risk ALL, died from multiorgan failure after high doses of methotrexate and ARA-C.
  
  
  CONCLUSIONS
  Patients with Down syndrome diagnosed with acute leukemia show a higher incidence of treatment-related complications, which affects their prognosis. Consequently, individualized treatment of these children in qualified units is essential.
authors.affiliation: Sección de Oncohematología, Servicio de Pediatría, Hospital Universitario Niño Jesús, Madrid, Spain. jsevilla.hnjs@salud.madrid.org
authors.forename: S
authors.initials: S
authors.lastname: Fernández-Plaza
authors.forename: J
authors.initials: J
authors.lastname: Sevilla
authors.forename: T
authors.initials: T
authors.lastname: Contra
authors.forename: N
authors.initials: N
authors.lastname: Martín
authors.forename: L
authors.initials: L
authors.lastname: Madero
mesh_terms: D002648:Child; D002675:Child, Preschool; D004314:Down Syndrome / Q000150:complications*; D005260:Female; D006801:Humans; D007223:Infant; D007947:Leukemia, Megakaryoblastic, Acute / Q000150:complications* / Q000401:mortality / Q000628:therapy; D008297:Male; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000150:complications* / Q000401:mortality / Q000628:therapy