Prognostic impact of expression of CD2, CD25, and/or CD30 in/on mast cells in systemic mastocytosis: a registry study of the European Competence Network on Mastocytosis
Expression of CD2, CD25 and/or CD30 in extracutaneous mast cells (MC) is a minor diagnostic criterion for systemic mastocytosis (SM) in the classification of the World Health Organization and International Consensus Classification. So far, it remains unknown whether expression of these antigens on M...
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| Hauptverfasser: | , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , |
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| Dokumenttyp: | Article (Journal) |
| Sprache: | Englisch |
| Veröffentlicht: |
March 2025
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| In: |
Leukemia
Year: 2025, Jahrgang: 39, Heft: 3, Pages: 675683 |
| ISSN: | 14765551 |
| DOI: | 10.1038/s41375-024-02504-3 |
| Online-Zugang: | Verlag, kostenfrei, Volltext: https://doi.org/10.1038/s41375-024-02504-3 Verlag, kostenfrei, Volltext: https://www.nature.com/articles/s41375-024-02504-3 
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| Verfasserangaben: | Axel Rüfer, Henning Nilius, Olivier Hermine, Marek Niedoszytko, Joanne N. G. Oude Elberink, Patrizia Bonadonna, Khalid Shoumariyeh, Theo Gulen, Karin Hartmann, Vito Sabato, Irena Angelova-Fischer, Daniel Baffoe, Deborah Christen, Anna Belloni Fortina, Christine Breynaert, Knut Brockow, Nikolas von Bubnoff, Horia Bumbea, Paul van Daele, Michael Doubek, Ingunn Dybedal, Chiara Elena, Christos Fokoloros, Aleksandra Górska, Marc Heizmann, Madlen Jentzsch, Saskia Klein, Johannes Lübke, Mattias Mattsson, André Mulder, Jens Panse, Tanja Daniela Schug, Mariarita Sciumè, Alex Stefan, Marlena Sztormowska, Judit Várkonyi, Friederike Wortmann, Akif Selim Yavuz, Martina Sperr, Jason Gotlib, Andreas Reiter, Massimo Triggiani, Wolfgang R. Sperr and Peter Valent |
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| 245 | 1 | 0 | |a Prognostic impact of expression of CD2, CD25, and/or CD30 in/on mast cells in systemic mastocytosis |b a registry study of the European Competence Network on Mastocytosis |c Axel Rüfer, Henning Nilius, Olivier Hermine, Marek Niedoszytko, Joanne N. G. Oude Elberink, Patrizia Bonadonna, Khalid Shoumariyeh, Theo Gulen, Karin Hartmann, Vito Sabato, Irena Angelova-Fischer, Daniel Baffoe, Deborah Christen, Anna Belloni Fortina, Christine Breynaert, Knut Brockow, Nikolas von Bubnoff, Horia Bumbea, Paul van Daele, Michael Doubek, Ingunn Dybedal, Chiara Elena, Christos Fokoloros, Aleksandra Górska, Marc Heizmann, Madlen Jentzsch, Saskia Klein, Johannes Lübke, Mattias Mattsson, André Mulder, Jens Panse, Tanja Daniela Schug, Mariarita Sciumè, Alex Stefan, Marlena Sztormowska, Judit Várkonyi, Friederike Wortmann, Akif Selim Yavuz, Martina Sperr, Jason Gotlib, Andreas Reiter, Massimo Triggiani, Wolfgang R. Sperr and Peter Valent |
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| 520 | |a Expression of CD2, CD25 and/or CD30 in extracutaneous mast cells (MC) is a minor diagnostic criterion for systemic mastocytosis (SM) in the classification of the World Health Organization and International Consensus Classification. So far, it remains unknown whether expression of these antigens on MC is of prognostic significance in SM. We performed a retrospective multi-center study of patients with SM using the data set of the registry of the European Competence Network on Mastocytosis, including 5034 patients with various MC disorders. The percentage of CD2-, CD25+ and/or CD30+ MC was considerably lower in patients with indolent SM compared to patients with advanced SM, including aggressive SM and MC leukemia. Whereas CD25 and CD30 expression in MC could not be associated with prognosis, we found that lack of CD2 expression in MC is associated with a significantly reduced overall survival (OS) in patients with SM (p < 0.0001). Lack of CD2 was also associated with the presence of extramedullary involvement affecting the spleen, liver, and/or lymph nodes (odds ratio 2.63 compared to SM with CD2+ MC). Together, lack of CD2 expression in MC is a prognostic marker and indicator of reduced OS and extramedullary disease expansion in patients with SM. | ||
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