TY - JOUR
T1 - Prognostic impact of eosinophils in mastocytosis
T2 - analysis of 2350 patients collected in the ECNM Registry
AU - Kluin-Nelemans, Hanneke C.
AU - Reiter, Andreas
AU - Illerhaus, Anja
AU - van Anrooij, Bjorn
AU - Hartmann, Karin
AU - Span, Lambertus F.R.
AU - Gorska, Aleksandra
AU - Niedoszytko, Marek
AU - Lange, Magdalena
AU - Scaffidi, Luigi
AU - Zanotti, Roberta
AU - Bonadonna, Patrizia
AU - Perkins, Cecelia
AU - Elena, Chiara
AU - Malcovati, Luca
AU - Shoumariyeh, Khalid
AU - von Bubnoff, Nikolas
AU - Parente, Roberta
AU - Triggiani, Massimo
AU - Schwaab, Juliana
AU - Jawhar, Mohamad
AU - Caroppo, Francesca
AU - Fortina, Anna Belloni
AU - Brockow, Knut
AU - Zink, Alexander
AU - Fuchs, David
AU - Kilbertus, Alex
AU - Yavuz, Akif Selim
AU - Doubek, Michael
AU - Mattsson, Mattias
AU - Hagglund, Hans
AU - Panse, Jens
AU - Sabato, Vito
AU - Aberer, Elisabeth
AU - Niederwieser, Dietger
AU - Breynaert, Christine
AU - Várkonyi, Judit
AU - Kennedy, Vanessa
AU - Lortholary, Olivier
AU - Jakob, Thilo
AU - Hermine, Olivier
AU - Rossignol, Julien
AU - Arock, Michel
AU - Gotlib, Jason
AU - Valent, Peter
AU - Sperr, Wolfgang R.
PY - 2019/1/1
Y1 - 2019/1/1
N2 - 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 (0.5–1.5 × 109/l), and 3.1% hypereosinophilia (HE; >1.5 × 109/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.
AB - 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 (0.5–1.5 × 109/l), and 3.1% hypereosinophilia (HE; >1.5 × 109/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.
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U2 - 10.1038/s41375-019-0632-4
DO - 10.1038/s41375-019-0632-4
M3 - Article
C2 - 31740811
AN - SCOPUS:85075382939
JO - Leukemia
JF - Leukemia
SN - 0887-6924
ER -