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Prognosis in pediatric hematologic malignancies is associated with serum concentration of mannose‐binding lectin‐associated serine protease‐2 (MASP‐2)
Author(s) -
Zehnder Aina,
Fisch Urs,
Hirt Andreas,
Niggli Felix K.,
Simon Arne,
Ozsahin Hulya,
Schlapbach Luregn J.,
Ammann Roland A.
Publication year - 2009
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.22028
Subject(s) - medicine , mannan binding lectin , hazard ratio , proportional hazards model , gastroenterology , lectin pathway , hematology , oncology , immunology , lectin , complement system , confidence interval , alternative complement pathway , antibody
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. Pediatr Blood Cancer 2009;53:53–57. © 2009 Wiley‐Liss, Inc.