Standardized indolent systemic mastocytosis evaluations across a health care system: implications for screening accuracy
Open accessAbstract
ABSTRACT: Timely diagnosis of systemic mastocytosis (SM) remains challenging because of care heterogeneity. We implemented a standardized approach for SM screening and diagnosis using a novel health care system-wide international screening registry. A retrospective analysis assessed rates of SM, cutaneous mastocytosis (CM), and molecular diagnoses before and 2 years after care standardization. The accuracy of individual and combined SM screening tests, basal serum tryptase (BST) ≥11.5 and ≥20.0 ng/mL, REMA ≥2, monomorphic maculopapular CM (MPCM), and elevated BST based upon tryptase genotype, was analyzed. Tryptase genotyping and high-sensitivity KIT p.D816V testing increased substantially 2 years after care standardization. SM diagnoses doubled from 47 to 94, and KIT p.D816V molecular diagnoses increased from 24 to 79. Mean BST and KIT p.D816V variant allele frequency values were significantly lower in patients diagnosed after standardization. Hereditary-alpha tryptasemia prevalence was increased in SM before care standardization (4/30 [13.3%]) but reflected the general population prevalence 2 years later at (5/76 [6.6%]). Elevated BST based upon genotype and BST ≥11.5 ng/mL had the highest sensitivities at 84.2% and 88.3%, respectively. The presence of monomorphic MPCM, elevated BST based upon tryptase genotype, and the combination of REMA ≥2 with elevated BST based upon tryptase genotype had specificities >90%. BST >20.0 ng/mL had low sensitivity and specificity and was not required to establish any indolent SM (ISM) diagnosis. Care standardization increased SM diagnosis rates, particularly in patients with low BSTs. Stratifying BST based upon genotype had the best overall sensitivity and specificity of any ISM screening test and improved the REMA score specificity.
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In a recent retrospective study, sensitivity of diagnostic assays for KIT D816V was higher for BST ≥11.5 ng/ml and BST elevated based on genotype, while specificity was highest for BST elevated based on genotype.
“In a recent retrospective study, the sensitivity of diagnostic assays for KIT D816V was further refined according to specific BST thresholds (≥11.5 ng/ml, ≥20 ng/ml and BST elevated due to HαT genotype) and REMA score ≥ 2. Sensitivity of PB testing was higher for BST ≥ 11.5 ng/ml and BST elevated based on genotype, while specificity was highest for BST elevated based on genotype (48).”
From Defining “Normal” basal serum tryptase levels: a context-dependent approach to improve diagnostics in systemic mastocytosis (2025) · cites this paper as [48]
Patients with a low REMA score (i.e., <2), no clear mediator-related symptoms (also according to other predictive scores), and elevated basal serum tryptase alone could benefit from an initial evaluation of the KIT D816V variant in peripheral blood, along with investigation of the hereditary alpha-tryptasemia genotype.
“In this context, patients with a low REMA score (i.e., <2), no clear mediator-related symptoms (also according to the other predictive scores), and elevated BST alone could benefit from an initial evaluation of the KIT D816V variant in PB, along with the investigation of the HαT genotype (48).”
From Defining “Normal” basal serum tryptase levels: a context-dependent approach to improve diagnostics in systemic mastocytosis (2025) · cites this paper as [48]
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- Authors
- McMurray, J. C., Pacheco, C. S., Schornack, B. J., Sun, X., Brunader, J. A., Scott, A. E., Ariza, J. S., Kou, C.-T. J., Costantino, R. C., Pittman, L. M., Adams, K. E., Waters, A. M., Pryor, E. M., Lyons, J. J., Metcalfe, D. D., Maric, I., & Boggs, N. A.
- Affiliations
- Walter Reed National Military Medical Center (US)
- Brooke Army Medical Center (US)
- National Institutes of Health (US)
- Defense Health Agency (US)
- 7Enterprise Intelligence and Data Solutions Program Office, Program Executive Office, Defense Healthcare Management Systems, San Antonio, TX
- Wilford Hall Ambulatory Surgical Center (US)
- University of California San Diego (US)
- 3Hematology Section, Department of Laboratory Medicine, National Institutes of Health, Bethesda, MD
- Uniformed Services University of the Health Sciences (US)
- Corresponding author
- Boggs, Nathan A.
- ORCID
- Venue
- Blood
- Publisher
- American Society of Hematology
- Published
- 2024-07-25
- Volume
- 144
- Issue
- 4
- Pages
- 408-419
- ISSN
- 0006-4971 (ISSN-L)
- 1528-0020
- PMC
- PMC11418066
- OpenAlex
- W4394933746
- Cited by
- 11
- References
- 56
- Citations by year
- 2026: 2 · 2025: 7 · 2024: 2
- FWCI
- 1.83
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- Blood (publishedVersion)
- Europe PMC (PubMed Central) (publishedVersion)
- PubMed (publishedVersion)
- PubMed Central (publishedVersion)
- Funders
- National Institute of Allergy and Infectious Diseases (SI2 AI138586, R00AI138586)
- Topics
- Life Sciences › Immunology and Microbiology › Immunology › Mast cells and histamine
- Health Sciences › Medicine › Gastroenterology › Gastrointestinal Tumor Research and Treatment
- Health Sciences › Medicine › Rheumatology › Eosinophilic Disorders and Syndromes
- Keywords
- indolent systemic mastocytosis, systemic mastocytosis, cutaneous mastocytosis, standardization of care, hereditary alpha-tryptasemia, diagnostic accuracy, screening registry
- MeSH terms
- Adult, Aged, Delivery of Health Care, Female, Genotype, Humans, Male, Mass Screening, Middle Aged, Retrospective Studies, Proto-Oncogene Proteins c-kit, Mastocytosis, Systemic, Tryptases
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
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