TY - JOUR
T1 - Prognostic impact of expression of CD2, CD25, and/or CD30 in/on mast cells in systemic mastocytosis
T2 - a registry study of the European Competence Network on Mastocytosis
AU - Rüfer, Axel
AU - Nilius, Henning
AU - Hermine, Olivier
AU - Niedoszytko, Marek
AU - Oude Elberink, Joanne N G
AU - Bonadonna, Patrizia
AU - Shoumariyeh, Khalid
AU - Gulen, Theo
AU - Hartmann, Karin
AU - Sabato, Vito
AU - Angelova-Fischer, Irena
AU - Baffoe, Daniel
AU - Christen, Deborah
AU - Belloni Fortina, Anna
AU - Breynaert, Christine
AU - Brockow, Knut
AU - von Bubnoff, Nikolas
AU - Bumbea, Horia
AU - van Daele, Paul
AU - Doubek, Michael
AU - Dybedal, Ingunn
AU - Elena, Chiara
AU - Fokoloros, Christos
AU - Górska, Aleksandra
AU - Heizmann, Marc
AU - Jentzsch, Madlen
AU - Klein, Saskia
AU - Lübke, Johannes
AU - Mattsson, Mattias
AU - Mulder, André
AU - Panse, Jens
AU - Schug, Tanja Daniela
AU - Sciumè, Mariarita
AU - Stefan, Alex
AU - Sztormowska, Marlena
AU - Várkonyi, Judit
AU - Wortmann, Friederike
AU - Yavuz, Akif Selim
AU - Sperr, Martina
AU - Gotlib, Jason
AU - Reiter, Andreas
AU - Triggiani, Massimo
AU - Sperr, Wolfgang R
AU - Valent, Peter
N1 - © 2025. The Author(s).
PY - 2025/3
Y1 - 2025/3
N2 - 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.
AB - 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.
U2 - 10.1038/s41375-024-02504-3
DO - 10.1038/s41375-024-02504-3
M3 - Article
C2 - 39815050
SN - 0887-6924
VL - 39
SP - 675
EP - 683
JO - Leukemia
JF - Leukemia
ER -