Kluin-Nelemans, Hanneke C., Reiter, Andreas, Illerhaus, Anja, van Anrooij, Bjorn, Hartmann, Karin ORCID: 0000-0002-4595-8226, Span, Lambertus F. R., Gorska, Aleksandra ORCID: 0000-0001-6682-1555, Niedoszytko, Marek ORCID: 0000-0003-1089-1911, Lange, Magdalena ORCID: 0000-0002-6967-1393, 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 ORCID: 0000-0001-5791-0775, Brockow, Knut, Zink, Alexander ORCID: 0000-0001-9313-6588, Fuchs, David, Kilbertus, Alex, Yavuz, Akif Selim, Doubek, Michael, Mattsson, Mattias ORCID: 0000-0002-9510-8801, Hagglund, Hans, Panse, Jens, Sabato, Vito, Aberer, Elisabeth, Niederwieser, Dietger, Breynaert, Christine ORCID: 0000-0002-7368-1068, Varkonyi, Judit, Kennedy, Vanessa, Lortholary, Olivier, Jakob, Thilo, Hermine, Olivier, Rossignol, Julien, Arock, Michel, Gotlib, Jason, Valent, Peter ORCID: 0000-0003-0456-5095 and Sperr, Wolfgang R. (2020). Prognostic impact of eosinophils in mastocytosis: analysis of 2350 patients collected in the ECNM Registry. Leukemia, 34 (4). S. 1090 - 1102. LONDON: SPRINGERNATURE. ISSN 1476-5551

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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.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Kluin-Nelemans, Hanneke C.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Reiter, AndreasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Illerhaus, AnjaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
van Anrooij, BjornUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hartmann, KarinUNSPECIFIEDorcid.org/0000-0002-4595-8226UNSPECIFIED
Span, Lambertus F. R.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gorska, AleksandraUNSPECIFIEDorcid.org/0000-0001-6682-1555UNSPECIFIED
Niedoszytko, MarekUNSPECIFIEDorcid.org/0000-0003-1089-1911UNSPECIFIED
Lange, MagdalenaUNSPECIFIEDorcid.org/0000-0002-6967-1393UNSPECIFIED
Scaffidi, LuigiUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Zanotti, RobertaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bonadonna, PatriziaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Perkins, CeceliaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Elena, ChiaraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Malcovati, LucaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Shoumariyeh, KhalidUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
von Bubnoff, NikolasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Parente, RobertaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Triggiani, MassimoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schwaab, JulianaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Jawhar, MohamadUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Caroppo, FrancescaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Fortina, Anna BelloniUNSPECIFIEDorcid.org/0000-0001-5791-0775UNSPECIFIED
Brockow, KnutUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Zink, AlexanderUNSPECIFIEDorcid.org/0000-0001-9313-6588UNSPECIFIED
Fuchs, DavidUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kilbertus, AlexUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Yavuz, Akif SelimUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Doubek, MichaelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Mattsson, MattiasUNSPECIFIEDorcid.org/0000-0002-9510-8801UNSPECIFIED
Hagglund, HansUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Panse, JensUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Sabato, VitoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Aberer, ElisabethUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Niederwieser, DietgerUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Breynaert, ChristineUNSPECIFIEDorcid.org/0000-0002-7368-1068UNSPECIFIED
Varkonyi, JuditUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kennedy, VanessaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Lortholary, OlivierUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Jakob, ThiloUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hermine, OlivierUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Rossignol, JulienUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Arock, MichelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gotlib, JasonUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Valent, PeterUNSPECIFIEDorcid.org/0000-0003-0456-5095UNSPECIFIED
Sperr, Wolfgang R.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-339396
DOI: 10.1038/s41375-019-0632-4
Journal or Publication Title: Leukemia
Volume: 34
Number: 4
Page Range: S. 1090 - 1102
Date: 2020
Publisher: SPRINGERNATURE
Place of Publication: LONDON
ISSN: 1476-5551
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
MAST-CELL DISEASE; SYSTEMIC MASTOCYTOSIS; C-KIT; ACTIVATING MUTATION; CLASSIFICATION; DISORDERS; MANIFESTATIONS; EXPRESSION; LEUKEMIAMultiple languages
Oncology; HematologyMultiple languages
URI: http://kups.ub.uni-koeln.de/id/eprint/33939

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