https://phaidra.vetmeduni.ac.at/o:3264 Serum chemistry profiling and prognostication in systemic mastocytosis: a registry-based study of the ECNM and GREM Lübke, Johannes Johannes Lübke 0000-0003-0767-1504 Heidelberg University Schwaab, Juliana Juliana Schwaab Heidelberg University Reiter, Andreas Andreas Reiter Heidelberg University Valent, Peter Peter Valent 0000-0003-0456-5095 Medical University of Vienna Sperr, Wolfgang R. Wolfgang R. Sperr Medical University of Vienna Panse, Jens Jens Panse 0000-0001-6316-3112 RWTH Aachen University / Center for Integrated Oncology Kluin-Nelemans, Hanneke C. Hanneke C. Kluin-Nelemans 0000-0003-2617-9427 University of Groningen Arock, Michel Michel Arock University Paris Hermine, Olivier Olivier Hermine University Paris Gotlib, Jason Jason Gotlib Stanford University Triggiani, Massimo Massimo Triggiani 0000-0001-7318-2093 University of Salerno, Hartmann, Karin Karin Hartmann 0000-0002-4595-8226 University of Basel Schug, Tanja Tanja Schug University Hospital Graz Sabato, Vito Vito Sabato University of Antwerp Doubek, Michael Michael Doubek Masaryk University Yavuz, Akif Selim Akif Selim Yavuz University of Istanbul Leven, Toon Toon Leven University Hospitals Leuven Breynaert, Christine Christine Breynaert 0000-0002-7368-1068 University Hospitals Leuven Zink, Alexander Alexander Zink 0000-0001-9313-6588 Technical University of Munich Brockow, Knut Knut Brockow 0000-0002-2775-3681 Technical University of Munich Caroppo, Francesca Francesca Caroppo 0000-0003-3583-0816 University of Padova Fortina, Anna Belloni Anna Belloni Fortina University of Padova Varkonyi, Judit Judit Varkonyi Varkonyi Parente, Roberta Roberta Parente University of Salerno Mattsson, Mattias Mattias Mattsson Uppsala University Hagglund, Hans Hans Hagglund Uppsala University Malcovati, Luca Luca Malcovati 0000-0002-1460-1611 Fondazione I.R.C.C.S. Policlinico San Matteo Elena, Chiara Chiara Elena Fondazione I.R.C.C.S. Policlinico San Matteo Perkins, Cecelia Cecelia Perkins Stanford University Müller, Sabine Sabine Müller University of Freiburg von Bubnoff, Nikolas Nikolas von Bubnoff University of Freiburg / German Cancer Consortium Shoumariyeh, Khalid Khalid Shoumariyeh University of Freiburg / German Cancer Consortium Bonadonna, Patrizia Patrizia Bonadonna Verona University Hospital Bonifacio, Massimiliano Massimiliano Bonifacio Verona University Hospital Zanotti, Roberta Roberta Zanotti Verona University Hospital Angelova-Fischer, Irena Irena Angelova-Fischer Johannes Kepler University Stefan, Alex Alex Stefan Johannes Kepler University Hadzijusufovic, Emir Emir Hadzijusufovic 0000-0001-7409-4204 University of Veterinary Medicine Vienna / Medical University of Vienna Karoline V, Karoline V. Karoline V. Karoline V Medical University of Vienna Lange, Magdalena Magdalena Lange 0000-0002-6967-1393 Medical University of Gdańsk Gorska, Aleksandra Aleksandra Gorska Medical University of Gdańsk Niedoszytko, Marek Marek Niedoszytko Medical University of Gdańsk Schmid, Alicia Alicia Schmid Heidelberg University Christen, Deborah Deborah Christen 0000-0003-2839-5983 RWTH Aachen University / Center for Integrated Oncology Oude Elberink, Hanneke N. G. Hanneke N. G. Oude Elberink 0000-0003-2844-2196 University of Groningen Span, Lambert F. R. Lambert F. R. Span University of Groningen Elsevier text eng 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. Humans; Mastocytosis, Systemicdiagnosisblood; Prognosis; Registries; Male; Female; Middle Aged; Adult; Aged; Biomarkersblood; Tryptasesblood http://creativecommons.org/licenses/by-nc-nd/4.0/ https://phaidra.vetmeduni.ac.at/api/object/o:3264/download 10.1182/bloodadvances.2024012756 https://phaidra.vetmeduni.ac.at/o:605 application/pdf Blood Advances 8(11), 2890-2900 (2024) Blood Advances 8 11 2890 2900 1.69 MB 2024