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Prognostic impact of eosinophils in mastocytosis: analysis of 2350 patients collected in the ECNM Registry

analysis of 2350 patients collected in the ECNM Registry
Authors: Kluin-Nelemans, Hanneke C.; Reiter, Andreas; Illerhaus, Anja; van Anrooij, Bjorn; Hartmann, Karin; Span, Lambertus F.R.; Gorska, Aleksandra; +39 Authors

Prognostic impact of eosinophils in mastocytosis: analysis of 2350 patients collected in the ECNM Registry

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 (0.5-1.5 × 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.

Keywords

EXPRESSION, Adult, Male, Adolescent, DISORDERS, Immunology, 610, CLASSIFICATION, Young Adult, 3211 Oncology and carcinogenesis, Eosinophilia, Humans, 1112 Oncology and Carcinogenesis, MAST-CELL DISEASE, ACTIVATING MUTATION, Child, Aged, Aged, 80 and over, LEUKEMIA Author Information, Science & Technology, 3202 Clinical sciences, Infant, Newborn, Infant, 1103 Clinical Sciences, Hematology, SYSTEMIC MASTOCYTOSIS, Middle Aged, Prognosis, C-KIT, Eosinophils, Survival Rate, MANIFESTATIONS, Oncology, Child, Preschool, Female, Human medicine, 3201 Cardiovascular medicine and haematology, Life Sciences & Biomedicine, LEUKEMIA, Mastocytosis, Follow-Up Studies

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    popularity
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    Top 10%
    influence
    This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
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    impulse
    This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
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selected citations
These citations are derived from selected sources.
This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Citations provided by BIP!
popularity
This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.
BIP!Popularity provided by BIP!
influence
This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Influence provided by BIP!
impulse
This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
BIP!Impulse provided by BIP!
43
Top 10%
Top 10%
Top 10%
Green
hybrid