Unlocking Prognosis in Systemic Mastocytosis: Insights from the ECNM and GREM Registry Study

Discover the groundbreaking insights from a registry-based study by the ECNM and GREM on how serum chemistry profiling can revolutionize prognosis and treatment strategies in systemic mastocytosis.
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Serum chemistry profiling and prognostication in systemic mastocytosis: a registry-based study of the ECNM and GREM.

Lübke et al., Blood Adv 2024
<!– DOI: 10.1182/bloodadvances.2024012756 //–>
https://doi.org/10.1182/bloodadvances.2024012756

This study highlights the significance of serum chemistry parameters in diagnosing and prognosticating systemic mastocytosis (SM) and its subvariants. Analyzing data from 3182 patients across the European Competence Network on Mastocytosis (ECNM) and the German Registry on Eosinophils and Mast Cells (GREM), the research identifies tryptase as the most specific serum parameter for SM screening and diagnosis. For distinguishing between indolent SM and advanced SM (AdvSM), parameters such as tryptase, alkaline phosphatase, ß2-microglobulin, lactate dehydrogenase (LDH), albumin, vitamin B12, and C-reactive protein proved most relevant. Notably, an elevated LDH ≥260U/L was linked to multi-lineage expansion and the presence of an associated myeloid neoplasm, while the highest tryptase levels were observed in mast cell leukemia (MCL). A novel three-tier risk classification system based on lactate dehydrogenase, ß2-microglobulin, and albumin levels demonstrated high predictive value for patient outcomes. This system further refined risk stratification within existing IPSM-AdvSM1/2 scores. The findings underscore the critical role of serum chemistry in the clinical management of SM, offering a more nuanced approach to diagnosis and risk assessment.

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