title: Acute leukemias in children with Down syndrome.
abstract: Children with Down syndrome have an increased risk for developing both acute myeloid as well as lymphoblastic leukemia. These leukemias differ in presenting characteristics and underlying biology when compared with leukemias occurring in non-Down syndrome children. Myeloid leukemia in children with Down syndrome is preceded by a preleukemic clone (transient leukemia or transient myeloproliferative disorder), which may disappear spontaneously, but may also need treatment in case of severe symptoms. Twenty percent of children with transient leukemia subsequently develop myeloid leukemia. This transition offers a unique model to study the stepwise development of leukemia, and of gene dosage effects mediated by aneuploidy.
authors.affiliation: Department of Pediatric Oncology/Hematology, Erasmus MC/Sophia Children's Hospital, Dr Molewaterplein 60, Rotterdam, The Netherlands. c.m.zwaan@erasmusmc.nl
authors.forename: Michel C
authors.initials: MC
authors.lastname: Zwaan
authors.forename: Dirk
authors.initials: D
authors.lastname: Reinhardt
authors.forename: Johann
authors.initials: J
authors.lastname: Hitzler
authors.forename: Paresh
authors.initials: P
authors.lastname: Vyas
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D002986:Clinical Trials as Topic; D018450:Disease Progression; D004314:Down Syndrome / Q000150:complications* / Q000235:genetics; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007951:Leukemia, Myeloid / Q000188:drug therapy / Q000235:genetics*; D009154:Mutation; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000188:drug therapy / Q000235:genetics*