Mannan-binding lectin deficiency attenuates acute gvhd in pediatric hematopoietic stem cell transplantation

Mannan-binding lectin deficiency attenuates acute gvhd in pediatric hematopoietic stem cell transplantation

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Access through your institution Buy or subscribe Mannan-binding lectin (MBL) is an acute phase protein that is synthesized in the liver and involved in first-line immune defense. Together


with MBL-associated serine proteases (MASPs), MBL mediates opsonization and activates the lectin pathway of C'. The MBL gene is highly polymorphic and specific single-nucleotide


polymorhism (SNP) patterns define at least 7 common haplotypes, giving 28 genotype possibilities. Exon-1 and/or promoter SNPs affect MBL serum levels and/or function (reviewed in ref. 1).


Particular SNPs and/or low serum levels are listed among common human immunodeficiencies but only rarely translate into a severe clinical phenotype.1 Nonetheless, in hematopoietic stem cell


transplantation (HSCT), a setting that _per se_ causes a transient but profound state of immunodeficiency, low MBL levels or particular MBL genotypes are reported to contribute to and


increase the rate of transplant-related infections. In consideration of the so far inconsistent findings of the role of MBL in HSCT in adult,2, 3, 4, 5 as well as pediatric6 HSCT, we


prospectively investigated the impact of MBL levels, genotypes and MASP-2 levels in a series of 99 pediatric (_n_=96) and young adult (_n_=3) HSCT recipients. MBL deficiency was defined by


MBL levels <300 ng/mL and/or particular MBL haplotypes. Patient characteristics, MBL genotype data and corresponding MBL levels are summarized in the Supplementary Information together


with MASP-2 serum levels and statistical analyses. This is a preview of subscription content, access via your institution ACCESS OPTIONS Access through your institution Subscribe to this


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support REFERENCES * Heitzeneder S, Seidel M, Forster-Waldl E, Heitger A . Mannan-binding lectin deficiency—good news, bad news, doesn't matter? _Clin Immunol_ 2012; 143: 22–38. Article


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references ACKNOWLEDGEMENTS We express our gratitude to the nurses of the stem cell transplantation unit and outpatient clinic for collecting the samples and, most of all, to the patients


and their parents for supporting the study by giving their informed consent and donating blood. This work was supported in part by a grant from the Austrian National Bank (#13075) and by an


Institutional Grant of the CCRI. It is dedicated to our friend, colleague and mentor—AH, who planned and supervised this study and who unexpectedly passed away after finalization of the


manuscript. AUTHOR INFORMATION Author notes * S Heitzeneder Present address: Pediatric Oncology Branch, National Cancer Institute, Bldg 10-CRC, R 1W-3840, 10 Center Dr, Bethesda, MD 20892,


USA, AUTHORS AND AFFILIATIONS * Transplantation Immunology, Children’s Cancer Research Institute, Vienna, Austria S Heitzeneder, U Pötschger, M Hölzl, D Heja, O A Haas & A Heitger *


medgen.at GmbH, Vienna, Austria P Zeitlhofer & O A Haas * St. Anna Kinderspital, Medical University Vienna, Vienna, Austria E Nowak, M G Seidel, A Lawitschka, E Förster-Waldl, S


Matthes-Martin, O A Haas & A Heitger * Department of Pediatrics, Medical University Vienna, Vienna, Austria E Förster-Waldl Authors * S Heitzeneder View author publications You can also


search for this author inPubMed Google Scholar * P Zeitlhofer View author publications You can also search for this author inPubMed Google Scholar * U Pötschger View author publications You


can also search for this author inPubMed Google Scholar * E Nowak View author publications You can also search for this author inPubMed Google Scholar * M G Seidel View author publications


You can also search for this author inPubMed Google Scholar * M Hölzl View author publications You can also search for this author inPubMed Google Scholar * A Lawitschka View author


publications You can also search for this author inPubMed Google Scholar * E Förster-Waldl View author publications You can also search for this author inPubMed Google Scholar * S


Matthes-Martin View author publications You can also search for this author inPubMed Google Scholar * D Heja View author publications You can also search for this author inPubMed Google


Scholar * O A Haas View author publications You can also search for this author inPubMed Google Scholar * A Heitger View author publications You can also search for this author inPubMed 


Google Scholar CORRESPONDING AUTHOR Correspondence to S Heitzeneder. ETHICS DECLARATIONS COMPETING INTERESTS The authors declare no conflict of interest. ADDITIONAL INFORMATION Supplementary


Information accompanies this paper on Bone Marrow Transplantation website SUPPLEMENTARY INFORMATION SUPPLEMENTARY INFORMATION (DOC 147 KB) RIGHTS AND PERMISSIONS Reprints and permissions


ABOUT THIS ARTICLE CITE THIS ARTICLE Heitzeneder, S., Zeitlhofer, P., Pötschger, U. _et al._ Mannan-binding lectin deficiency attenuates acute GvHD in pediatric hematopoietic stem cell


transplantation. _Bone Marrow Transplant_ 50, 1127–1129 (2015). https://doi.org/10.1038/bmt.2015.81 Download citation * Published: 11 May 2015 * Issue Date: August 2015 * DOI:


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