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Serum chemistry profiling and prognostication in systemic mastocytosis: a registry-based study of the ECNM and GREM
J. Lübke, A. Schmid, D. Christen, HNG. Oude Elberink, LFR. Span, M. Niedoszytko, A. Gorska, M. Lange, KV. Gleixner, E. Hadzijusufovic, A. Stefan, I. Angelova-Fischer, R. Zanotti, M. Bonifacio, P. Bonadonna, K. Shoumariyeh, N. von Bubnoff, S....
Jazyk angličtina Země Spojené státy americké
Typ dokumentu časopisecké články
NLK
Directory of Open Access Journals
od 2016
PubMed Central
od 2016
Europe PubMed Central
od 2016
ROAD: Directory of Open Access Scholarly Resources
od 2016
- MeSH
- biologické markery krev MeSH
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- prognóza MeSH
- registrace * MeSH
- senioři MeSH
- systémová mastocytóza * diagnóza krev MeSH
- tryptasy krev MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
Certain laboratory abnormalities correlate with subvariants of systemic mastocytosis (SM) and are often prognostically relevant. To assess the diagnostic and prognostic value of individual serum chemistry parameters in SM, 2607 patients enrolled within the European Competence Network on Mastocytosis and 575 patients enrolled within the German Registry on Eosinophils and Mast Cells were analyzed. For screening and diagnosis of SM, tryptase was identified as the most specific serum parameter. For differentiation between indolent and advanced SM (AdvSM), the following serum parameters were most relevant: tryptase, alkaline phosphatase, β2-microglobulin, lactate dehydrogenase (LDH), albumin, vitamin B12, and C-reactive protein (P < .001). With regard to subvariants of AdvSM, an elevated LDH of ≥260 U/L was associated with multilineage expansion (leukocytosis, r = 0.37, P < .001; monocytosis, r = 0.26, P < .001) and the presence of an associated myeloid neoplasm (P < .001), whereas tryptase levels were highest in mast cell leukemia (MCL) vs non-MCL (308μg/L vs 146μg/L, P = .003). Based on multivariable analysis, the hazard-risk weighted assignment of 1 point to LDH (hazard ratio [HR], 2.1; 95% confidence interval [CI], 1.1-4.0; P = .018) and 1.5 points each to β2-microglobulin (HR, 2.7; 95% CI, 1.4-5.4; P = .004) and albumin (HR, 3.3; 95% CI, 1.7-6.5; P = .001) delineated a highly predictive 3-tier risk classification system (0 points, 8.1 years vs 1 point, 2.5 years; ≥1.5 points, 1.7 years; P < .001). Moreover, serum chemistry parameters enabled further stratification of patients classified as having an International Prognostic Scoring System for Mastocytosis-AdvSM1/2 risk score (P = .027). In conclusion, serum chemistry profiling is a crucial tool in the clinical practice supporting diagnosis and prognostication of SM and its subvariants.
Allergy Unit Verona University Hospital Verona Italy
Center for Integrated Oncology Aachen Bonn Cologne Düsseldorf Aachen Germany
Department of Allergology Medical University of Gdańsk Gdańsk Poland
Department of Biomedicine University Hospital Basel and University of Basel Basel Switzerland
Department of Dermatology and Venereology University Hospital Graz Graz Austria
Department of Dermatology Medical Center Faculty of Medicine University of Freiburg Freiburg Germany
Department of Dermatology Venereology and Allergology Medical University of Gdańsk Gdańsk Poland
Department of Hematology Semmelweis University Budapest Hungary
Department of Medicine Pediatric Dermatology Unit University of Padova Padova Italy
Department of Medicine Section of Hematology Verona University Hospital Verona Italy
Division of Allergy and Clinical Immunology University of Salerno Salerno Italy
Division of Hematology Department of Medical Sciences Uppsala University Uppsala Sweden
Division of Hematology Istanbul Medical School University of Istanbul Istanbul Turkey
German Cancer Consortium Heidelberg Germany
Hematology Unit Fondazione IRCCS Policlinico San Matteo Pavia Italy
Ludwig Boltzmann Institute for Hematology and Oncology Medical University of Vienna Vienna Austria
Citace poskytuje Crossref.org
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