Clinical features and survival of patients with indolent systemic mastocytosis defined by the updated WHO classification
The result's identifiers
Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F65269705%3A_____%2F20%3A00072922" target="_blank" >RIV/65269705:_____/20:00072922 - isvavai.cz</a>
Alternative codes found
RIV/00216224:14110/20:00114918
Result on the web
<a href="https://onlinelibrary.wiley.com/doi/full/10.1111/all.14248" target="_blank" >https://onlinelibrary.wiley.com/doi/full/10.1111/all.14248</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.1111/all.14248" target="_blank" >10.1111/all.14248</a>
Alternative languages
Result language
angličtina
Original language name
Clinical features and survival of patients with indolent systemic mastocytosis defined by the updated WHO classification
Original language description
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.
Czech name
—
Czech description
—
Classification
Type
J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database
CEP classification
—
OECD FORD branch
30102 - Immunology
Result continuities
Project
Result was created during the realization of more than one project. More information in the Projects tab.
Continuities
P - Projekt vyzkumu a vyvoje financovany z verejnych zdroju (s odkazem do CEP)
Others
Publication year
2020
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů
Data specific for result type
Name of the periodical
Allergy
ISSN
0105-4538
e-ISSN
—
Volume of the periodical
75
Issue of the periodical within the volume
8
Country of publishing house
US - UNITED STATES
Number of pages
12
Pages from-to
1927-1938
UT code for WoS article
000552392800006
EID of the result in the Scopus database
—