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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

A. Rüfer, H. Nilius, O. Hermine, M. Niedoszytko, JNG. Oude Elberink, P. Bonadonna, K. Shoumariyeh, T. Gulen, K. Hartmann, V. Sabato, I. Angelova-Fischer, D. Baffoe, D. Christen, A. Belloni Fortina, C. Breynaert, K. Brockow, N. von Bubnoff, H....

. 2025 ; 39 (3) : 675-683. [pub] 20250115

Jazyk angličtina Země Anglie, Velká Británie

Typ dokumentu časopisecké články, multicentrická studie

Perzistentní odkaz   https://www.medvik.cz/link/bmc25009681

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.

Allergy Unit Azienda Ospedaliera Universitaria Integrata di Verona Verona Italy

Brno University Hospital Brno Czech Republic

Center for Integrated Oncology Aachen Bonn Cologne Düsseldorf Aachen Germany and Department of Haematology Oncology Haemostaseology and Stem Cell Transplantation University Hospital RWTH Aachen Aachen Germany

Department for Hematology and Oncology Universitätsklinikum Schleswig Holstein and University Cancer Center Schleswig Holstein Lübeck Germany

Department of Allergology Medical University of Gdansk Gdansk Poland

Department of Clinical Chemistry University Hospital Bern Inselspital Bern Switzerland

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

Department of Hematology Bone Marrow Transplant Unit Carol Davila University of Medicine and Pharmacy Emergency University Hospital Bucharest Romania

Department of Hematology Luzerner Kantonsspital University of Luzern Luzern Switzerland

Department of Hematology Semmelweis University Budapest Hungary

Department of Hematology Uppsala University Hospital and Department of Immunology Genetics and Pathology Uppsala University Uppsala Sweden

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

Department of Internal Medicine Erasmus University Rotterdam Rotterdam The Netherlands

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

Department of Medicine Solna Division of Immunology and Allergy Karolinska Institutet Stockholm Sweden

Department of Respiratory Medicine and Allergy Karolinska University Hospital Huddinge Stockholm

Departments of Hematology and Pharmacology Oslo University Hospital Rikshospitalet Oslo Norway

Division of Allergy and Clinical Immunology University of Salerno Salerno Italy

Division of Hematology Istanbul Medical School University of Istanbul Istanbul Turkey

German Cancer Consortium Heidelberg Germany

Hôpital Necker Imagine Institute INSERM U1163 University of Sorbonne Paris Cité Centre national de référence des mastocytoses Paris France

Kantonsspital Aarau AG Medizinische Universitätsklinik Hämatologie Aarau Switzerland

Klinik für Dermatologie und Venerologie Kepler Universitätsklinikum Linz Austria

Klinik für Innere Medizin 3 Schwerpunkt Hämatologie und Onkologie Kepler Universitätsklinikum Linz Austria

Klinik und Poliklinik für Dermatologie und Allergologie am Biederstein

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

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

Mastocytosis Center Attikon University Hospital of Athens Athens Greece

Medical Clinic 1 Universitätsklinikum Leipzig AöR Leipzig Germany

Pediatric Dermatology Internal Medicine Azienda Ospedaliera Università di Padova Padova Italy

Policlinico San Matteo Pavia Fondazione IRCCS Pavia Italy

S C Ematologia Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Milan Italy

Stanford Cancer Institute Stanford University School of Medicine Stanford CA USA

Technische Universität München München Germany

Universitätsklinik für Dermatologie und Venerologie Medical University of Graz Graz Austria

Universitätsspital Basel Basel Switzerland

Universiteit Antwerpen Campus Drie Eiken Antwerp Belgium

University Medical Center Groningen University of Groningen Groningen The Netherlands

University of California San Francisco CA USA

Citace poskytuje Crossref.org

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