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.

Allergy Unit Verona University Hospital Verona Italy

Center for Integrated Oncology Aachen Bonn Cologne Düsseldorf Aachen Germany

Department of Allergology Medical University of Gdańsk Gdańsk Poland

Department of Allergology University Medical Center Groningen University of Groningen Groningen The Netherlands

Department of Biomedicine University Hospital Basel and University of Basel Basel Switzerland

Department of Dermatology and Allergy School of Medicine Technical University of Munich Munich Germany

Department of Dermatology and Venereology Allergy Center Kepler University Hospital Johannes Kepler University Linz Austria

Department of Dermatology and Venereology University Hospital Graz Graz Austria

Department of Dermatology Medical Center Faculty of Medicine University of Freiburg Freiburg Germany

Department of Dermatology Venereology and Allergology Medical University of Gdańsk Gdańsk Poland

Department of Hematology and Oncology Medical Center University of Schleswig Holstein Campus Lübeck Lübeck Germany

Department of Hematology and Oncology University Hospital Mannheim Heidelberg University Mannheim Germany

Department of Hematology Semmelweis University Budapest Hungary

Department of Hematology University Medical Center Groningen University of Groningen Groningen The Netherlands

Department of Immunology Allergology Rheumatology Faculty of Medicine and Health Sciences University of Antwerp and Antwerp University Hospital Antwerpen Belgium

Department of Internal Medicine Hematology and Oncology University Hospital Brno and Faculty of Medicine Masaryk University Brno Czech Republic

Department of Medicine 1 Medical Center University of Freiburg Faculty of Medicine University of Freiburg Freiburg Germany

Department of Medicine Pediatric Dermatology Unit University of Padova Padova Italy

Department of Medicine Section of Hematology Verona University Hospital Verona Italy

Department of Oncology Hematology Hemostaseology and Stem Cell Transplantation University Hospital RWTH Aachen Aachen Germany

Department University Clinic for Companion Animals and Horses Internal Medicine Small Animals University Clinic for Small Animals University of Veterinary Medicine Vienna Austria

Division of Allergy and Clinical Immunology University of Salerno Salerno Italy

Division of Allergy Department of Dermatology University Hospital Basel and University of Basel Basel Switzerland

Division of Hematology and Hemostaseology Department of Internal Medicine 1 Medical University of Vienna Vienna Austria

Division of Hematology Department of Medical Sciences Uppsala University Uppsala Sweden

Division of Hematology Istanbul Medical School University of Istanbul Istanbul Turkey

French Reference Center for Mastocytosis Hospital Necker Assistance Publique Hôpitaux de Paris Imagine Institute University Paris Descartes Paris France

French Reference Center for Mastocytosis Pitié Salpêtrière Hospital Assistance Publique Hôpitaux de Paris University Paris Sorbonne Paris France

German Cancer Consortium Heidelberg Germany

Hematology Unit Fondazione IRCCS Policlinico San Matteo Pavia Italy

KU Leuven Department of Microbiology Immunology and Transplantation Allergy and Clinical Immunology Research Group and MASTeL University Hospitals Leuven Leuven Belgium

Ludwig Boltzmann Institute for Hematology and Oncology Medical University of Vienna Vienna Austria

Stanford Cancer Institute Stanford University School of Medicine Stanford Cancer Institute Stanford CA

University Clinic for Hematology and Oncology Kepler University Hospital Johannes Kepler University Linz Austria

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