Cytogenetic and molecular aberrations and worse outcome for male patients in systemic mastocytosis

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F65269705%3A_____%2F21%3A00074226" target="_blank" >RIV/65269705:_____/21:00074226 - isvavai.cz</a>

Alternative codes found

RIV/00216224:14740/21:00121919

Result on the web

<a href="https://www.thno.org/v11p0292.htm" target="_blank" >https://www.thno.org/v11p0292.htm</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.7150/thno.51872" target="_blank" >10.7150/thno.51872</a>

Result language

angličtina

Original language name

Cytogenetic and molecular aberrations and worse outcome for male patients in systemic mastocytosis

Original language description

In systemic mastocytosis (SM), the clinical features and survival vary greatly. Patient-related factors determining the outcome in SM are largely unknown. Methods: We examined the impact of sex on the clinical features, progression-free survival (PFS), and overall survival (OS) in 3403 patients with mastocytosis collected in the registry of the European Competence Network on Mastocytosis (ECNM). The impact of cytogenetic and molecular genetic aberrations on sex differences was analyzed in a subset of patients. Results: Of all patients enrolled, 55.3% were females. However, a male predominance was found in a subset of advanced SM (AdvSM) patients, namely SM with an associated hematologic neoplasm (SM-AHN, 70%; p &lt; 0.001). Correspondingly, organomegaly (male: 23% vs. female: 13%, p = 0.007) was more, whereas skin involvement (male: 71% vs. female: 86%, p = 0.001) was less frequent in males. In all patients together, OS (p &lt; 0.0001) was significantly inferior in males, and also within the WHO sub-categories indolent SM, aggressive SM (ASM) and SM-AHN. PFS was significantly (p = 0.0002) worse in males when all patients were grouped together; due to low numbers of events, this significance persisted only in the subcategory smoldering SM. Finally, prognostically relevant cytogenetic abnormalities (10% vs. 5%, p = 0.006) or molecular aberrations (SRSF2/ASXLI/RUNXI profile; 63% vs. 40%, p = 0.003) were more frequently present in males. Conclusions: Male sex has a major impact on clinical features, disease progression, and survival in mastocytosis. Male patients have an inferior survival, which seems related to the fact that they more frequently develop a multi-mutated AdvSM associated with a high-risk molecular background.

Czech name

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Czech description

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Type

J_imp - Article in a specialist periodical, which is included in the Web of Science database

CEP classification

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OECD FORD branch

30204 - Oncology

Project

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Continuities

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Publication year

2021

Confidentiality

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Name of the periodical

Theranostics

ISSN

1838-7640

e-ISSN

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Volume of the periodical

11

Issue of the periodical within the volume

1

Country of publishing house

AU - AUSTRALIA

Number of pages

12

Pages from-to

292-303

UT code for WoS article

000582957500019

EID of the result in the Scopus database

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