Journalartikel
Autorenliste: Kluin-Nelemans, Hanneke C.; Reiter, Andreas; Illerhaus, Anja; van Anrooij, Bjorn; Hartmann, Karin; Span, Lambertus F. R.; Gorska, Aleksandra; Niedoszytko, Marek; Lange, Magdalena; Scaffidi, Luigi; Zanotti, Roberta; Bonadonna, Patrizia; Perkins, Cecelia; Elena, Chiara; Malcovati, Luca; Shoumariyeh, Khalid; von Bubnoff, Nikolas; Parente, Roberta; Triggiani, Massimo; Schwaab, Juliana; Jawhar, Mohamad; Caroppo, Francesca; Fortina, Anna Belloni; Brockow, Knut; Zink, Alexander; Fuchs, David; Kilbertus, Alex; Yavuz, Akif Selim; Doubek, Michael; Mattsson, Mattias; Hagglund, Hans; Panse, Jens; Sabato, Vito; Aberer, Elisabeth; Niederwieser, Dietger; Breynaert, Christine; Varkonyi, Judit; Kennedy, Vanessa; Lortholary, Olivier; Jakob, Thilo; Hermine, Olivier; Rossignol, Julien; Arock, Michel; Gotlib, Jason; Valent, Peter; Sperr, Wolfgang R.
Jahr der Veröffentlichung: 2020
Seiten: 1090-1101
Zeitschrift: Leukemia
Bandnummer: 34
Heftnummer: 4
ISSN: 0887-6924
eISSN: 1476-5551
DOI Link: https://doi.org/10.1038/s41375-019-0632-4
Verlag: Springer Nature [academic journals on nature.com]
Abstract:
Systemic mastocytosis (SM) is frequently associated with eosinophilia. To examine its prevalence and clinical impact in all WHO classification-based subcategories, we analyzed eosinophil counts in 2350 mastocytosis patients using the dataset of the European Competence Network on Mastocytosis. Ninety percent of patients had normal eosinophil counts, 6.8% mild eosinophilia (0.5-1.5x10(9)/l), and 3.1% hypereosinophilia (HE; >1.5x10(9)/l). Eosinophilia/HE were mainly present in patients with advanced SM (17%/19%), and only rarely recorded in patients with indolent and smoldering SM (5%/1%), and some patients with cutaneous mastocytosis. The eosinophil count correlated with organomegaly, dysmyelopoiesis, and the WHO classification, but not with mediator-related symptoms or allergy. Eosinophilia at diagnosis had a strong prognostic impact (p<0.0001) on overall survival (OS) and progression-free survival (PFS), with a 10-year OS of 19% for patients with HE, 70% for those with mild eosinophilia, and 88% for patients with normal eosinophil counts. In 89% of patients with follow-up data (n=1430, censored at start of cytoreductive therapy), eosinophils remained stable. In those with changing eosinophil counts (increase/decrease or mixed pattern), OS and PFS were inferior compared with patients with stable eosinophil counts. In conclusion, eosinophilia and HE are more prevalent in advanced SM and are predictors of a worse outcome.
Zitierstile
Harvard-Zitierstil: Kluin-Nelemans, H., Reiter, A., Illerhaus, A., van Anrooij, B., Hartmann, K., Span, L., et al. (2020) Prognostic impact of eosinophils in mastocytosis: analysis of 2350 patients collected in the ECNM Registry, Leukemia, 34(4), pp. 1090-1101. https://doi.org/10.1038/s41375-019-0632-4
APA-Zitierstil: Kluin-Nelemans, H., Reiter, A., Illerhaus, A., van Anrooij, B., Hartmann, K., Span, L., Gorska, A., Niedoszytko, M., Lange, M., Scaffidi, L., Zanotti, R., Bonadonna, P., Perkins, C., Elena, C., Malcovati, L., Shoumariyeh, K., von Bubnoff, N., Parente, R., Triggiani, M., ...Sperr, W. (2020). Prognostic impact of eosinophils in mastocytosis: analysis of 2350 patients collected in the ECNM Registry. Leukemia. 34(4), 1090-1101. https://doi.org/10.1038/s41375-019-0632-4
Schlagwörter
ACTIVATING MUTATION; C-KIT; LEUKEMIA; MAST-CELL DISEASE; SYSTEMIC MASTOCYTOSIS
