title: Resistance to activated protein C (APCR) in children with acute lymphoblastic leukaemia--the need for a prospective multicentre study.
abstract: Activated protein C resistance (APCR), usually due to the Arg506-->Gln point mutation of the factor V gene, has emerged as the most important hereditary cause of venous thromboembolism. Using an aPTT based method in the presence of APC, together with a DNA technique based on the polymerase chain reaction, we investigated 65 leukaemic children and 65 age-matched healthy controls for the presence of this mutation. In both groups three children showed APCR. All six children showed the common factor V gene mutation, Arg506-->Gln. Although no child in the control group presented with thrombosis, all three children with acute lymphoblastic leukaemia had thromboembolic events. Whether the poor anticoagulant response to activated protein C in leukaemic children treated with prednisone, vincristine, daunorubicin and asparaginase affects the risk of thrombotic events requires a more extensive multicentre study.
authors.affiliation: Department of Pediatrics, Westfälische Wilheims University, Frankfurt, Germany.
authors.forename: U
authors.initials: U
authors.lastname: Nowak-Göttl
authors.forename: I
authors.initials: I
authors.lastname: Aschka
authors.forename: H G
authors.initials: HG
authors.lastname: Koch
authors.forename: J
authors.initials: J
authors.lastname: Boos
authors.forename: B
authors.initials: B
authors.lastname: Dockhorn-Dworniczak
authors.forename: T
authors.initials: T
authors.lastname: Deufel
authors.forename: H
authors.initials: H
authors.lastname: Jürgens
authors.forename: B
authors.initials: B
authors.lastname: Kohlhase
authors.forename: N
authors.initials: N
authors.lastname: Kuhn
authors.forename: A
authors.initials: A
authors.lastname: Laupert
mesh_terms: D000293:Adolescent; D000990:Antithrombin III / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D005165:Factor V / Q000378:metabolism; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000503:physiopathology*; D011446:Prospective Studies; D011486:Protein C / Q000378:metabolism*; D017293:Protein S / Q000378:metabolism
chemical_list: D011486:Protein C; D017293:Protein S; D000990:Antithrombin III; D005165:Factor V