
<lom:lom xmlns:lom="https://oer-repo.uibk.ac.at/lom" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" xsi:schemaLocation="https://oer-repo.uibk.ac.at/lom/latest https://w3id.org/oerbase/profiles/lomuibk/latest/schemas/lom-uibk.xsd">
  
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<lom:catalog>phaidra.vetmeduni.ac.at</lom:catalog>

  
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<lom:langstring xml:lang="x-none">o:3264</lom:langstring>

  
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<lom:identifier>
  
<lom:catalog>DOI</lom:catalog>

  
<lom:entry>
  
<lom:langstring xml:lang="x-none">10.1182/bloodadvances.2024012756</lom:langstring>

  
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</lom:identifier>

  
<lom:title>
  
<lom:langstring xml:lang="en">Serum chemistry profiling and prognostication in systemic mastocytosis: a registry-based study of the ECNM and GREM</lom:langstring>

  
</lom:title>

  
<lom:description>
  
<lom:langstring xml:lang="en">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 &lt; .001). With regard to subvariants of AdvSM, an elevated LDH of ≥260 U/L was associated with multilineage expansion (leukocytosis, r = 0.37, P &lt; .001; monocytosis, r = 0.26, P &lt; .001) and the presence of an associated myeloid neoplasm (P &lt; .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 &lt; .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.</lom:langstring>

  
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<lom:language>eng</lom:language>

  
<lom:keyword>
  
<lom:langstring xml:lang="en">Humans; Mastocytosis, Systemicdiagnosisblood; Prognosis; Registries; Male; Female; Middle Aged; Adult; Aged; Biomarkersblood; Tryptasesblood</lom:langstring>

  
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<lom:datetime>2024-07-22T09:51:56.679Z</lom:datetime>

  
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VERSION:3.0
N:Lübke;Johannes;
FN:Johannes Lübke
X-ORCID:https://orcid.org/0000-0003-0767-1504
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N:Schwaab;Juliana;
FN:Juliana Schwaab
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VERSION:3.0
N:Reiter;Andreas;
FN:Andreas Reiter
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VERSION:3.0
N:Valent;Peter;
FN:Peter Valent
X-ORCID:https://orcid.org/0000-0003-0456-5095
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VERSION:3.0
N:Sperr;Wolfgang R.;
FN:Wolfgang R. Sperr
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VERSION:3.0
N:Panse;Jens;
FN:Jens Panse
X-ORCID:https://orcid.org/0000-0001-6316-3112
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VERSION:3.0
N:Kluin-Nelemans;Hanneke C.;
FN:Hanneke C. Kluin-Nelemans
X-ORCID:https://orcid.org/0000-0003-2617-9427
END:VCARD</lom:vcard>

  
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N:Arock;Michel;
FN:Michel Arock
END:VCARD</lom:vcard>

  
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N:Hermine;Olivier;
FN:Olivier Hermine
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N:Gotlib;Jason;
FN:Jason Gotlib
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N:Triggiani;Massimo;
FN:Massimo Triggiani
X-ORCID:https://orcid.org/0000-0001-7318-2093
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N:Hartmann;Karin;
FN:Karin Hartmann
X-ORCID:https://orcid.org/0000-0002-4595-8226
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N:Schug;Tanja;
FN:Tanja Schug
END:VCARD</lom:vcard>

  
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<lom:centity>
  
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VERSION:3.0
N:Sabato;Vito;
FN:Vito Sabato
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N:Doubek;Michael;
FN:Michael Doubek
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VERSION:3.0
N:Yavuz;Akif Selim;
FN:Akif Selim Yavuz
END:VCARD</lom:vcard>

  
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VERSION:3.0
N:Leven;Toon;
FN:Toon Leven
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N:Breynaert;Christine;
FN:Christine Breynaert
X-ORCID:https://orcid.org/0000-0002-7368-1068
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N:Zink;Alexander;
FN:Alexander Zink
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N:Brockow;Knut;
FN:Knut Brockow
X-ORCID:https://orcid.org/0000-0002-2775-3681
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VERSION:3.0
N:Caroppo;Francesca;
FN:Francesca Caroppo
X-ORCID:https://orcid.org/0000-0003-3583-0816
END:VCARD</lom:vcard>

  
</lom:centity>

  
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VERSION:3.0
N:Fortina;Anna Belloni;
FN:Anna Belloni Fortina
END:VCARD</lom:vcard>

  
</lom:centity>

  
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VERSION:3.0
N:Varkonyi;Judit;
FN:Judit Varkonyi
END:VCARD</lom:vcard>

  
</lom:centity>

  
<lom:centity>
  
<lom:vcard>BEGIN:VCARD
VERSION:3.0
N:Parente;Roberta;
FN:Roberta Parente
END:VCARD</lom:vcard>

  
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<lom:centity>
  
<lom:vcard>BEGIN:VCARD
VERSION:3.0
N:Mattsson;Mattias;
FN:Mattias Mattsson
END:VCARD</lom:vcard>

  
</lom:centity>

  
<lom:centity>
  
<lom:vcard>BEGIN:VCARD
VERSION:3.0
N:Hagglund;Hans;
FN:Hans Hagglund
END:VCARD</lom:vcard>

  
</lom:centity>

  
<lom:centity>
  
<lom:vcard>BEGIN:VCARD
VERSION:3.0
N:Malcovati;Luca;
FN:Luca Malcovati
X-ORCID:https://orcid.org/0000-0002-1460-1611
END:VCARD</lom:vcard>

  
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VERSION:3.0
N:Elena;Chiara;
FN:Chiara Elena
END:VCARD</lom:vcard>

  
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<lom:vcard>BEGIN:VCARD
VERSION:3.0
N:Perkins;Cecelia;
FN:Cecelia Perkins
END:VCARD</lom:vcard>

  
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VERSION:3.0
N:Müller;Sabine;
FN:Sabine Müller
END:VCARD</lom:vcard>

  
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N:von Bubnoff;Nikolas;
FN:Nikolas von Bubnoff
END:VCARD</lom:vcard>

  
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N:Shoumariyeh;Khalid;
FN:Khalid Shoumariyeh
END:VCARD</lom:vcard>

  
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VERSION:3.0
N:Bonadonna;Patrizia;
FN:Patrizia Bonadonna
END:VCARD</lom:vcard>

  
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<lom:centity>
  
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VERSION:3.0
N:Bonifacio;Massimiliano;
FN:Massimiliano Bonifacio
END:VCARD</lom:vcard>

  
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<lom:centity>
  
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VERSION:3.0
N:Zanotti;Roberta;
FN:Roberta Zanotti
END:VCARD</lom:vcard>

  
</lom:centity>

  
<lom:centity>
  
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VERSION:3.0
N:Angelova-Fischer;Irena;
FN:Irena Angelova-Fischer
END:VCARD</lom:vcard>

  
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<lom:centity>
  
<lom:vcard>BEGIN:VCARD
VERSION:3.0
N:Stefan;Alex;
FN:Alex Stefan
END:VCARD</lom:vcard>

  
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<lom:centity>
  
<lom:vcard>BEGIN:VCARD
VERSION:3.0
N:Hadzijusufovic;Emir;
FN:Emir Hadzijusufovic
X-ORCID:https://orcid.org/0000-0001-7409-4204
END:VCARD</lom:vcard>

  
</lom:centity>

  
<lom:centity>
  
<lom:vcard>BEGIN:VCARD
VERSION:3.0
N:Karoline V;Karoline V.;
FN:Karoline V. Karoline V
END:VCARD</lom:vcard>

  
</lom:centity>

  
<lom:centity>
  
<lom:vcard>BEGIN:VCARD
VERSION:3.0
N:Lange;Magdalena;
FN:Magdalena Lange
X-ORCID:https://orcid.org/0000-0002-6967-1393
END:VCARD</lom:vcard>

  
</lom:centity>

  
<lom:centity>
  
<lom:vcard>BEGIN:VCARD
VERSION:3.0
N:Gorska;Aleksandra;
FN:Aleksandra Gorska
END:VCARD</lom:vcard>

  
</lom:centity>

  
<lom:centity>
  
<lom:vcard>BEGIN:VCARD
VERSION:3.0
N:Niedoszytko;Marek;
FN:Marek Niedoszytko
END:VCARD</lom:vcard>

  
</lom:centity>

  
<lom:centity>
  
<lom:vcard>BEGIN:VCARD
VERSION:3.0
N:Schmid;Alicia;
FN:Alicia Schmid
END:VCARD</lom:vcard>

  
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<lom:centity>
  
<lom:vcard>BEGIN:VCARD
VERSION:3.0
N:Christen;Deborah;
FN:Deborah Christen
X-ORCID:https://orcid.org/0000-0003-2839-5983
END:VCARD</lom:vcard>

  
</lom:centity>

  
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<lom:vcard>BEGIN:VCARD
VERSION:3.0
N:Oude Elberink;Hanneke N. G.;
FN:Hanneke N. G. Oude Elberink
X-ORCID:https://orcid.org/0000-0003-2844-2196
END:VCARD</lom:vcard>

  
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<lom:vcard>BEGIN:VCARD
VERSION:3.0
N:Span;Lambert F. R.;
FN:Lambert F. R. Span
END:VCARD</lom:vcard>

  
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<lom:source>
  
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