Refined diagnostic criteria for bone marrow mastocytosis: a proposal of the European competence network on mastocytosis
Open accessAbstract
In the current classification of the World Health Organization (WHO), bone marrow mastocytosis (BMM) is a provisional variant of indolent systemic mastocytosis (ISM) defined by bone marrow involvement and absence of skin lesions. However, no additional diagnostic criteria for BMM have been proposed. Within the registry dataset of the European Competence Network on Mastocytosis, we compared characteristics and outcomes of 390 patients with BMM and 1175 patients with typical ISM. BMM patients were significantly older, predominantly male, had lower tryptase and lower burden of neoplastic mast cells, and displayed a higher frequency of allergic reactions, mainly triggered by Hymenoptera, than patients with typical ISM. The estimated 10-year progression-free survival of BMM and typical ISM was 95.9% and 92.6%, respectively. In BMM patients defined by WHO-based criteria, the presence of one B-Finding and tryptase level ≥125 ng/mL were identified as risk factors for progression in multivariate analyses. BMM patients without any of these risk factors were found to have better progression-free survival (p < 0.05) and better overall survival (p < 0.05) than other ISM patients. These data support the proposal to define BMM as a separate SM variant characterized by SM criteria, absence of skin lesions, absence of B-Findings, and tryptase levels <125 ng/mL.
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A normal BST value does not exclude a diagnosis of SM.
“although normal BST value does not exclude the diagnosis”
From Defining “Normal” basal serum tryptase levels: a context-dependent approach to improve diagnostics in systemic mastocytosis (2025) · cites this paper as [51]
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- Authors
- Zanotti, R., Bonifacio, M., Lucchini, G., Sperr, W. R., Scaffidi, L., van Anrooij, B., Oude Elberink, H. N., Rossignol, J., Hermine, O., Gorska, A., Lange, M., Hadzijusufovic, E., Miething, C., Müller, S., Perkins, C., Shomali, W., Elena, C., Illerhaus, A., Jawhar, M., Parente, R., Caroppo, F., Solomianyi, O., Zink, A., Mattsson, M., Yavuz, A. S., Panse, J., Varkonyi, J., Doubek, M., Sabato, V., Breynaert, C., Vucinic, V., Schug, T., Hägglund, H., Wortmann, F., Brockow, K., Angelova-Fischer, I., Belloni Fortina, A., Triggiani, M., Reiter, A., Hartmann, K., Malcovati, L., Gotlib, J., Shoumariyeh, K., Niedoszytko, M., Arock, M., Kluin-Nelemans, H. C., Bonadonna, P., & Valent, P.
- Affiliations
- University of Verona (IT)
- Ludwig Boltzmann Institute for Hematology and Oncology (AT)
- Centre National de la Recherche Scientifique (FR)
- Gdańsk Medical University (PL)
- University of Veterinary Medicine Vienna (AT)
- University of Freiburg (DE)
- Stanford Medicine (US)
- Policlinico San Matteo Fondazione (IT)
- University of Cologne (DE)
- Heidelberg University (DE)
- University of Salerno (IT)
- University of Padua (IT)
- Johannes Kepler University of Linz (AT)
- Technical University of Munich (DE)
- Uppsala University (SE)
- Istanbul University (TR)
- Universitätsklinikum Aachen (DE)
- Masaryk University (CZ)
- University of Antwerp (BE)
- Universitair Ziekenhuis Leuven (BE)
- University of Graz (AT)
- University of Lübeck (DE)
- University of Basel (CH)
- University of Pavia (IT)
- Pitié-Salpêtrière Hospital (FR)
- Azienda Ospedaliera Universitaria Integrata Verona (IT)
- Corresponding author
- Bonifacio, Massimiliano
- ORCID
- Zanotti, Roberta
- Bonifacio, Massimiliano
- Sperr, Wolfgang R.
- Rossignol, Julien
- Hermine, Olivier
- Lange, Magdalena
- Hadzijusufovic, Emir
- Miething, Cornelius
- Müller, Sabine
- Perkins, Cecelia
- Shomali, William
- Elena, Chiara
- Illerhaus, Anja
- Jawhar, Mohamad
- Parente, Roberta
- Caroppo, Francesca
- Zink, Alexander
- Mattsson, Mattias
- Yavuz, Akif Selim
- Panse, Jens
- Doubek, Michael
- Sabato, Vito
- Breynaert, Christine
- Hägglund, Hans
- Wortmann, Friederike
- Brockow, Knut
- Triggiani, Massimo
- Reiter, Andreas
- Hartmann, Karin
- Malcovati, Luca
- Gotlib, Jason
- Shoumariyeh, Khalid
- Niedoszytko, Marek
- Arock, Michel
- Bonadonna, Patrizia
- Valent, Peter
- Venue
- Leukemia
- Published
- 2021-09-20
- Volume
- 36
- Issue
- 2
- Pages
- 516-524
- ISSN
- 0887-6924 (ISSN-L)
- 1476-5551
- OpenAlex
- W3201033674
- Cited by
- 56
- References
- 38
- Citations by year
- 2026: 4 · 2025: 8 · 2024: 13 · 2023: 12 · 2022: 14 · 2021: 4
- FWCI
- 4.01
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Leukemia (publishedVersion)
- PubMed (publishedVersion)
- Kölner Universitäts PublikationsServer (Universität zu Köln) (publishedVersion)
- University of Groningen research database (University of Groningen / Centre for Information Technology) (publishedVersion, other-oa)
- Lirias (KU Leuven) (submittedVersion)
- mediaTUM – the media and publications repository of the Technical University Munich (Technical University Munich) (submittedVersion)
- RWTH Publications (RWTH Aachen) (submittedVersion)
- Padua Research Archive (University of Padova) (submittedVersion)
- University of Groningen research database (University of Groningen / Centre for Information Technology) (submittedVersion, other-oa)
- Funders
- Università degli Studi di Verona
- Topics
- Life Sciences › Immunology and Microbiology › Immunology › Mast cells and histamine
- Health Sciences › Medicine › Gastroenterology › Gastrointestinal Tumor Research and Treatment
- Health Sciences › Medicine › Physiology › Histiocytic Disorders and Treatments
- Keywords
- bone marrow mastocytosis, indolent systemic mastocytosis, diagnostic criteria, tryptase, progression-free survival, overall survival, risk factors for progression, allergic reactions, hymenoptera venom allergy
- MeSH terms
- Adult, Aged, Aged, 80 and over, Bone Marrow, Europe, Female, Follow-Up Studies, Humans, Male, Mast Cells, Mastocytosis, Middle Aged, Prognosis, Skin Diseases, Survival Rate, Mastocytosis, Systemic, Tryptases
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