https://ashpublications.org/bloodadvances/article/doi/10.1182/bloodadvances.2024012756/515667
https://pubmed.ncbi.nlm.nih.gov/38593217/
Blood Adv . 2024 Apr 9:bloodadvances.2024012756. doi: 10.1182/bloodadvances.2024012756. Online ahead of print.
Johannes Lübke, Alicia Schmid, Deborah Christen, Hanneke N.G. Oude Elberink, Lambert F.R. Span, Marek Niedoszytko, Aleksandra Górska, 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 G Hagglund, Mattias Mattsson, Roberta Parente, Judit Varkonyi, Anna Belloni Fortina, Francesca Caroppo, Knut Brockow, Alexander Zink, Christine Breynaert, Toon Ieven, Akif Selim Yavuz, Michael Doubek, Vito Sabato, Tanja Schug, Karin Hartmann, Massimo Triggiani, Jason Gotlib, Olivier Hermine, Michel Arock, Hanneke C. Kluin-Nelemans, Jens Peter Panse, Wolfgang R Sperr, Peter Valent, Andreas Reiter, Juliana Schwaab
Key Points
Serum chemistry profiling is of diagnostic and prognostic relevance based on easily accessible parameters.
Serum chemistry markers help to assess and quantify organ damage.
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 (ECNM) and 575 patients enrolled within the German Registry on Eosinophils and Mast Cells (GREM) 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 (AP), ß2-microglobulin, lactate dehydrogenase (LDH), albumin, vitamin B12, and C-reactive protein (P<0.001). With regard to subvariants of AdvSM, an elevated LDH ≥260U/L was associated with multi-lineage expansion (leukocytosis, r=0.37, P<0.001; monocytosis, r=0.26, P<0.001) and the presence of an associated myeloid neoplasm (P<0.001), whereas tryptase levels were highest in mast cell leukemia (MCL vs. non-MCL, 308 µg/L vs. 146 µg/L, P=0.003). Based on multivariable analysis, the hazard-risk weighted assignment of 1 point to lactate dehydrogenase (HR 2.1 [95% CI 1.1-4.0], P=0.018) and 1.5 points each to ß2-microglobulin (HR 2.7 [95% CI 1.4-5.4], P=0.004) and albumin (HR 3.3 [95% CI 1.7-6.5], P=0.001) delineated a highly predictive three-tier risk classification system (0 points, 8.1 years vs. 1 point, 2.5 years, ≥1.5 points, 1.7 years; P<0.001). Moreover, serum chemistry parameters enabled further stratification of IPSM-AdvSM1/2 risk-score classified patients (P=0.027). In conclusion, serum chemistry profiling is a crucial tool in the clinical practice supporting diagnosis and prognostication of SM and its subvariants.-
Subjects: Myeloid Neoplasia
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