Mastocytosis in the age of precision medicine

Summary Mastocytosis encompasses a spectrum of clonal mast cell disorders characterized by the proliferation and accumulation of atypical mast cells in various organs, including the skin, bone marrow, and gastrointestinal tract. Initially described in 1869 as a cutaneous manifestation, the systemic variant (SM), involving additional organs, was documented in 1949. Clinical distinctions are made between indolent SM (ISM), where mediator-related symptoms predominate, and more aggressive forms, in which organ dysfunction is the primary concern. In recent decades, there has been continuous progress in elucidating the pathogenesis, classification, and management of mastocytosis, aided by specialized networks such as the European Competence Network Mastocytosis (ECNM) and the German Competence Network on Mastocytosis (Kompetenznetzwerk Mastozytose e. V.). A significant therapeutic advancement has been the development of targeted tyrosine kinase inhibitors, including midostaurin and avapritinib, which have been utilized for several years in treating aggressive SM. Recently, avapritinib in lower dosage was also approved for ISM patients with moderate to severe symptoms. For patients with milder forms of ISM, a symptom-oriented basic therapy is recommended. The diagnosis of SM requires interdisciplinary collaboration and strict adherence to established diagnostic criteria. Moreover, innovative patient-centered approaches, such as the MASTHAVE® app, support ongoing follow-up and have the potential to enhance quality of life. Long-term research efforts are increasingly directed toward developing personalized therapies that target the molecular mechanisms underlying the disease.

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Publication Details

Journal
Allergo Journal International
Published
2025-04-03
DOI
https://doi.org/10.1007/s40629-025-00327-x
Citations
1
Primary Topic
Mast cells and histamine
Type
article
Field-Weighted Citation Impact
0.80
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Mastocytosis in the age of precision medicine

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Allergo Journal International
Mast cells and histamine
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Mastocytosis in the age of precision medicine

Peter Schmid‐Grendelmeier, Torsten Zuberbier, Deborah Christen, Jens Panse, Nicola Wagner, Knut Brockow, Norbert Mülleneisen, Undine Lippert, Claudia Lang, Petra Staubach, Franziska Ruëff, Friederike Bärhold, Frank Siebenhaar, Sabine Altrichter, Randolf Brehler, Nikolas C. C. von Bubnoff, Karin Hartmann, Polina Pyatilova, Marcus Joest, Dagmar von Bubnoff, Marcus Maurer, Hagen Ott, Ludger Klimek, Stefani Röseler, Kristin aufm Kampe
article en
1 citations

Abstract

Summary Mastocytosis encompasses a spectrum of clonal mast cell disorders characterized by the proliferation and accumulation of atypical mast cells in various organs, including the skin, bone marrow, and gastrointestinal tract. Initially described in 1869 as a cutaneous manifestation, the systemic variant (SM), involving additional organs, was documented in 1949. Clinical distinctions are made between indolent SM (ISM), where mediator-related symptoms predominate, and more aggressive forms, in which organ dysfunction is the primary concern. In recent decades, there has been continuous progress in elucidating the pathogenesis, classification, and management of mastocytosis, aided by specialized networks such as the European Competence Network Mastocytosis (ECNM) and the German Competence Network on Mastocytosis (Kompetenznetzwerk Mastozytose e. V.). A significant therapeutic advancement has been the development of targeted tyrosine kinase inhibitors, including midostaurin and avapritinib, which have been utilized for several years in treating aggressive SM. Recently, avapritinib in lower dosage was also approved for ISM patients with moderate to severe symptoms. For patients with milder forms of ISM, a symptom-oriented basic therapy is recommended. The diagnosis of SM requires interdisciplinary collaboration and strict adherence to established diagnostic criteria. Moreover, innovative patient-centered approaches, such as the MASTHAVE® app, support ongoing follow-up and have the potential to enhance quality of life. Long-term research efforts are increasingly directed toward developing personalized therapies that target the molecular mechanisms underlying the disease.

Allergo Journal InternationalVol. 34(3)
Johannes Kepler University of Linz (AT), Johannes Gutenberg University Mainz (DE), AO Foundation (CH), University Hospital of Basel (CH), LMU Klinikum (DE), Humboldt-Universität zu Berlin (DE), Kinderkrankenhaus auf der Bult (DE), Universitätsklinikum Erlangen (DE), Düsseldorf University Hospital (DE), University Medical Center of the Johannes Gutenberg University Mainz (DE), Integrated Oncology (United States) (US), University Hospital of Zurich (CH), University Hospital Münster (DE), Fraunhofer Institute for Translational Medicine and Pharmacology (DE), University Hospital Schleswig-Holstein (DE), Universitätsmedizin Göttingen (DE), Kepler Universitätsklinikum (AT), Zentrum für Rhinologie und Allergologie (DE), Department of Biomedicine Basel (CH), Nederlands Astmacentrum Davos (CH), Helios Kliniken (DE), Klinikum Leverkusen (DE), Hochgebirgsklinik Davos (CH), Heinrich Heine University Düsseldorf (DE), Technical University of Munich (DE), Ludwig-Maximilians-Universität München (DE), RWTH Aachen University (DE), University of Lübeck (DE)
Openalex Percentile: Top 30%
Mast cells and histamine
0.80
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