Most cited article - PubMed ID 36868470
European Competence Network on Mastocytosis (ECNM): 20-Year Jubilee, Updates, and Future Perspectives
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.
- MeSH
- Ki-1 Antigen * metabolism MeSH
- CD2 Antigens * metabolism MeSH
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Mast Cells * metabolism immunology pathology MeSH
- Survival Rate MeSH
- Adolescent MeSH
- Young Adult MeSH
- Prognosis MeSH
- Interleukin-2 Receptor alpha Subunit * metabolism MeSH
- Registries MeSH
- Retrospective Studies MeSH
- Aged, 80 and over MeSH
- Aged MeSH
- Mastocytosis, Systemic * mortality metabolism diagnosis pathology MeSH
- Check Tag
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Adolescent MeSH
- Young Adult MeSH
- Male MeSH
- Aged, 80 and over MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Multicenter Study MeSH
- Geographicals
- Europe MeSH
- Names of Substances
- Ki-1 Antigen * MeSH
- CD2 Antigens * MeSH
- IL2RA protein, human MeSH Browser
- Interleukin-2 Receptor alpha Subunit * MeSH
