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Serum chemistry profiling and prognostication in systemic mastocytosis: a registry-based study of the ECNM and GREM

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...

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Main Authors: Lübke, Johannes (Author) , Schmid, Alicia (Author) , Christen, Deborah (Author) , Oude Elberink, Hanneke N. G. (Author) , Span, Lambert F. R. (Author) , Niedoszytko, Marek (Author) , Gorska, Aleksandra (Author) , Lange, Magdalena (Author) , Gleixner, Karoline V. (Author) , Hadzijusufovic, Emir (Author) , Stefan, Alex (Author) , Angelova-Fischer, Irena (Author) , Zanotti, Roberta (Author) , Bonifacio, Massimiliano (Author) , Bonadonna, Patrizia (Author) , Shoumariyeh, Khalid (Author) , von Bubnoff, Nikolas (Author) , Müller, Sabine (Author) , Perkins, Cecelia (Author) , Elena, Chiara (Author) , Malcovati, Luca (Author) , Hagglund, Hans (Author) , Mattsson, Mattias (Author) , Parente, Roberta (Author) , Varkonyi, Judit (Author) , Fortina, Anna Belloni (Author) , Caroppo, Francesca (Author) , Brockow, Knut (Author) , Zink, Alexander (Author) , Breynaert, Christine (Author) , Leven, Toon (Author) , Yavuz, Akif Selim (Author) , Doubek, Michael (Author) , Sabato, Vito (Author) , Schug, Tanja (Author) , Hartmann, Karin (Author) , Triggiani, Massimo (Author) , Gotlib, Jason (Author) , Hermine, Olivier (Author) , Arock, Michel (Author) , Kluin-Nelemans, Hanneke C. (Author) , Panse, Jens (Author) , Sperr, Wolfgang R. (Author) , Valent, Peter (Author) , Reiter, Andreas (Author) , Schwaab, Juliana (Author)
Format: Article (Journal)
Language:English
Published: June 11 2024
In: Blood advances
Year: 2024, Volume: 8, Issue: 11, Pages: 2890-2900
ISSN:2473-9537
DOI:10.1182/bloodadvances.2024012756
Online Access:Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1182/bloodadvances.2024012756
Verlag, lizenzpflichtig, Volltext: https://ashpublications.org/bloodadvances/article/8/11/2890/515667/Serum-chemistry-profiling-and-prognostication-in
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Author Notes:Johannes Lübke, Alicia Schmid, Deborah Christen, Hanneke N. G. Oude Elberink, Lambert F. R. Span, Marek Niedoszytko, Aleksandra Gorska, Magdalena Lange, Karoline V. Gleixner, Emir Hadzijusufovic, Alex Stefan, Irena Angelova-Fischer, Roberta Zanotti, Massimiliano Bonifacio, Patrizia Bonadonna, Khalid Shoumariyeh, Nikolas von Bubnoff, Sabine Müller, Cecelia Perkins, Chiara Elena, Luca Malcovati, Hans Hagglund, Mattias Mattsson, Roberta Parente, Judit Varkonyi, Anna Belloni Fortina, Francesca Caroppo, Knut Brockow, Alexander Zink, Christine Breynaert, Toon Leven, Akif Selim Yavuz, Michael Doubek, Vito Sabato, Tanja Schug, Karin Hartmann, Massimo Triggiani, Jason Gotlib, Olivier Hermine, Michel Arock, Hanneke C. Kluin-Nelemans, Jens Panse, Wolfgang R. Sperr, Peter Valent, Andreas Reiter and Juliana Schwaab

MARC

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520 |a 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. 
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