title: Prognosis in pediatric hematologic malignancies is associated with serum concentration of mannose-binding lectin-associated serine protease-2 (MASP-2).
abstract: BACKGROUND
  Mannose-binding lectin (MBL) and MBL-associated serine protease-2 (MASP-2) are key components of the lectin pathway of complement activation. Their serum concentrations show a wide interindividual variability. This study investigated whether the concentration of MBL and MASP-2 is associated with prognosis in pediatric patients with cancer.
  
  
  METHODS
  In this retrospective multicenter study, MBL and MASP-2 were measured by commercially available ELISA in frozen remnants of serum taken at diagnosis. Associations of overall survival (OS) and event-free survival (EFS) with MBL and MASP-2 were assessed by multivariate Cox regression accounting for prognostically relevant clinical variables.
  
  
  RESULTS
  In the 372 patients studied, median serum concentration of MBL was 2,808 microg/L (range, 2-10,060) and 391 microg/L (46-2,771) for MASP-2. The estimated 4-year EFS was 0.60 (OS, 0.78). In the entire, heterogeneous sample, MBL and MASP-2 were not significantly associated with OS or EFS. In patients with hematologic malignancies, however, higher MASP-2 was associated with better EFS in a significant and clinically relevant way (hazard ratio per tenfold increase (HR), 0.22; 95% CI, 0.09-0.54; P = 0.001). This was due to patients with lymphoma (HR, 0.11; 95% CI, 0.03-0.47; P = 0.003), but less for those with acute leukemia (HR, 0.35; 95% CI, 0.11-1.15; P = 0.083).
  
  
  CONCLUSIONS
  In this study, higher MASP-2 was associated with better EFS in pediatric patients with hematologic malignancies, especially lymphoma. Whether MASP-2 is an independent prognostic factor affecting risk stratification and anticancer therapy needs to be assessed in prospective, disease-specific studies.
authors.affiliation: Department of Pediatrics, University of Bern, Bern, Switzerland.
authors.forename: Aina
authors.initials: A
authors.lastname: Zehnder
authors.forename: Urs
authors.initials: U
authors.lastname: Fisch
authors.forename: Andreas
authors.initials: A
authors.lastname: Hirt
authors.forename: Felix K
authors.initials: FK
authors.lastname: Niggli
authors.forename: Arne
authors.initials: A
authors.lastname: Simon
authors.forename: Hulya
authors.initials: H
authors.lastname: Ozsahin
authors.forename: Luregn J
authors.initials: LJ
authors.lastname: Schlapbach
authors.forename: Roland A
authors.initials: RA
authors.lastname: Ammann