Prognosis in pediatric hematologic malignancies is associated with serum concentration of mannose-binding lectin-associated serine protease-2 (MASP-2)

Zehnder, Aina, Fisch, Urs, Hirt, Andreas, Niggli, Felix K., Simon, Arne, Ozsahin, Hulya, Schlapbach, Luregn J. and Ammann, Roland A. (2009) Prognosis in pediatric hematologic malignancies is associated with serum concentration of mannose-binding lectin-associated serine protease-2 (MASP-2). Pediatric Blood and Cancer, 53 1: 53-57. doi:10.1002/pbc.22028


Author Zehnder, Aina
Fisch, Urs
Hirt, Andreas
Niggli, Felix K.
Simon, Arne
Ozsahin, Hulya
Schlapbach, Luregn J.
Ammann, Roland A.
Title Prognosis in pediatric hematologic malignancies is associated with serum concentration of mannose-binding lectin-associated serine protease-2 (MASP-2)
Journal name Pediatric Blood and Cancer   Check publisher's open access policy
ISSN 1545-5009
1545-5017
Publication date 2009-07-15
Sub-type Article (original research)
DOI 10.1002/pbc.22028
Volume 53
Issue 1
Start page 53
End page 57
Total pages 611
Place of publication Hoboken, NJ, United States
Publisher John Wiley & Sons
Language eng
Formatted 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 μg/L (range, 2-10,060) and 391 μg/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).

Conclusion: 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. 
Keyword Cancer
Innate immunity
Mannose-binding lectin (MBL)
MBL-associated serine protease-2 (MASP-2)
Prognosis
Risk group assignment
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status Non-UQ

Document type: Journal Article
Sub-type: Article (original research)
Collection: School of Medicine Publications
 
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