title: The course of fibrinolytic proteins in children with malignant bone tumours.
abstract: To evaluate the role of fibrinolytic and proteolytic proteins in children and adolescents suffering from Ewing sarcoma or osteosarcoma with respect to postoperative complications and late outcome, a prospective two-arm two-centre study was conducted. Plasminogen, plasminogen activator inhibitor (PAI)-1, tissue-type plasminogen activator (t-PA) and urokinase plasminogen activator (u-PA) were investigated in the pre-surgical period and in the postoperative follow-up period in children suffering from Ewing sarcoma (ES; n = 36) or osteosarcoma (OS; n = 39). In addition, the factor V mutation (FV) Q506, protein C, protein S, antithrombin and lipoprotein (a) were determined. All children received LMWH (EnoxaparinR) 1 mg/kg s.c. once daily over a period of 6 weeks to 3 months. Besides a short-lasting increase of PAI-1 in patients with OS on day 1 and in children with Es on day 14, a small and significant but clinically irrelevant difference was found on days 7-10 for plasminogen, t-PA and u-PA. No thromboembolic complications occurred in patients treated with LMWH and having a prothrombotic genetic risk factor. Within one year of surgery 7 out of 36 patients with ES and 5 out of 39 children with OS showed a relapse of their disease. Prior to the first local tumour therapy, 5 out of 7 children with ES and relapse had elevated u-PA concentrations compared with 2 out of 5 children in the OS group. No such differences were found for PAI-1- or t-PA antigen.
  
  
  CONCLUSIONS
  The role of u-PA as a possible follow-up marker for a poorer outcome in children with ES should be evaluated in a prospective multicentre study.
authors.affiliation: Paediatric Haematology/Oncology, University Hospital, Münster, Germany.
authors.forename: U
authors.initials: U
authors.lastname: Nowak-Göttl
authors.forename: N
authors.initials: N
authors.lastname: Münchow
authors.forename: U
authors.initials: U
authors.lastname: Klippel
authors.forename: M
authors.initials: M
authors.lastname: Paulussen
authors.forename: S
authors.initials: S
authors.lastname: Bielack
authors.forename: K
authors.initials: K
authors.lastname: Ullrich
authors.forename: S
authors.initials: S
authors.lastname: Ehrenforth
mesh_terms: D000293:Adolescent; D001859:Bone Neoplasms / Q000097:blood* / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D012516:Osteosarcoma / Q000097:blood* / Q000601:surgery; D010958:Plasminogen / Q000378:metabolism*; D017395:Plasminogen Activator Inhibitor 1 / Q000097:blood*; D011183:Postoperative Complications; D011446:Prospective Studies; D012512:Sarcoma, Ewing / Q000097:blood* / Q000601:surgery; D012697:Serine Endopeptidases / Q000097:blood*; D010959:Tissue Plasminogen Activator / Q000097:blood; D016896:Treatment Outcome; D014568:Urokinase-Type Plasminogen Activator / Q000097:blood
chemical_list: D017395:Plasminogen Activator Inhibitor 1; D010958:Plasminogen; D012697:Serine Endopeptidases; D010959:Tissue Plasminogen Activator; D014568:Urokinase-Type Plasminogen Activator