Trizuljak, Jakub, Sperr, Wolfgang R., Nekvindova, Lucie ORCID: 0000-0002-8866-2310, Elberink, Hanneke O., Gleixner, Karoline, V, Gorska, Aleksandra, Lange, Magdalena ORCID: 0000-0002-6967-1393, Hartmann, Karin ORCID: 0000-0002-4595-8226, Illerhaus, Anja ORCID: 0000-0003-0521-203X, Bonifacio, Massimiliano, Perkins, Cecelia, Elena, Chiara, Malcovati, Luca, Fortina, Anna B., Shoumariyeh, Khalid, Jawhar, Mohamad, Zanotti, Roberta, Bonadonna, Patrizia, Caroppo, Francesca, Zink, Alexander ORCID: 0000-0001-9313-6588, Triggiani, Massimo, Parente, Roberta, von Bubnoff, Nikolas, Yavuz, Akif S., Hagglund, Hans, Mattsson, Mattias ORCID: 0000-0002-9510-8801, Panse, Jens, Jaekel, Nadja, Kilbertus, Alex, Hermine, Olivier, Arock, Michel, Fuchs, David, Sabato, Vito, Brockow, Knut, Bretterklieber, Agnes, Niedoszytko, Marek ORCID: 0000-0003-1089-1911, van Anrooij, Bjorn, Reiter, Andreas, Gotlib, Jason, Kluin-Nelemans, Hanneke C., Mayer, Jiri, Doubek, Michael and Valent, Peter ORCID: 0000-0003-0456-5095 (2020). Clinical features and survival of patients with indolent systemic mastocytosis defined by the updated WHO classification. Allergy, 75 (8). S. 1927 - 1939. HOBOKEN: WILEY. ISSN 1398-9995

Full text not available from this repository.

Abstract

Background: In indolent systemic mastocytosis (ISM), several risk factors of disease progression have been identified. Previous studies, performed with limited patient numbers, have also shown that the clinical course in ISM is stable and comparable to that of cutaneous mastocytosis (CM). The aim of this project was to compare the prognosis of patients with ISM with that of patients with CM. Methods: We employed a dataset of 1993 patients from the registry of the European Competence Network on Mastocytosis (ECNM) to compare outcomes of ISM and CM. Results: We found that overall survival (OS) is worse in ISM compared to CM. Moreover, in patients with typical ISM, bone marrow mastocytosis (BMM), and smoldering SM (SSM), 4.1% of disease progressions have been observed (4.9% of progressions in typical ISM group, 1.7% in BMM, and 9.4% in SSM). Progressions to advanced SM were observed in 2.9% of these patients. In contrast, six patients with CM (1.7%) converted to ISM and no definitive progression to advanced SM was found. No significant differences in OS and event-free survival (EFS) were found when comparing ISM, BMM, and SSM. Higher risk of both progression and death was significantly associated with male gender, worse performance status, and organomegaly. Conclusion: Our data confirm the clinical impact of the WHO classification that separates ISM from CM and from other SM variants.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Trizuljak, JakubUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Sperr, Wolfgang R.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Nekvindova, LucieUNSPECIFIEDorcid.org/0000-0002-8866-2310UNSPECIFIED
Elberink, Hanneke O.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gleixner, Karoline, VUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gorska, AleksandraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Lange, MagdalenaUNSPECIFIEDorcid.org/0000-0002-6967-1393UNSPECIFIED
Hartmann, KarinUNSPECIFIEDorcid.org/0000-0002-4595-8226UNSPECIFIED
Illerhaus, AnjaUNSPECIFIEDorcid.org/0000-0003-0521-203XUNSPECIFIED
Bonifacio, MassimilianoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Perkins, CeceliaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Elena, ChiaraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Malcovati, LucaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Fortina, Anna B.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Shoumariyeh, KhalidUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Jawhar, MohamadUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Zanotti, RobertaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bonadonna, PatriziaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Caroppo, FrancescaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Zink, AlexanderUNSPECIFIEDorcid.org/0000-0001-9313-6588UNSPECIFIED
Triggiani, MassimoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Parente, RobertaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
von Bubnoff, NikolasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Yavuz, Akif S.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hagglund, HansUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Mattsson, MattiasUNSPECIFIEDorcid.org/0000-0002-9510-8801UNSPECIFIED
Panse, JensUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Jaekel, NadjaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kilbertus, AlexUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hermine, OlivierUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Arock, MichelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Fuchs, DavidUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Sabato, VitoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Brockow, KnutUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bretterklieber, AgnesUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Niedoszytko, MarekUNSPECIFIEDorcid.org/0000-0003-1089-1911UNSPECIFIED
van Anrooij, BjornUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Reiter, AndreasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gotlib, JasonUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kluin-Nelemans, Hanneke C.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Mayer, JiriUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Doubek, MichaelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Valent, PeterUNSPECIFIEDorcid.org/0000-0003-0456-5095UNSPECIFIED
URN: urn:nbn:de:hbz:38-325116
DOI: 10.1111/all.14248
Journal or Publication Title: Allergy
Volume: 75
Number: 8
Page Range: S. 1927 - 1939
Date: 2020
Publisher: WILEY
Place of Publication: HOBOKEN
ISSN: 1398-9995
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
MAST-CELL DISEASE; TRYPTASE LEVELS; MUTATIONS; DIAGNOSIS; PATTERNS; UTILITY; BURDEN; ADULTSMultiple languages
Allergy; ImmunologyMultiple languages
URI: http://kups.ub.uni-koeln.de/id/eprint/32511

Downloads

Downloads per month over past year

Altmetric

Export

Actions (login required)

View Item View Item