Kluin-Nelemans, Hanneke C., Jawhar, Mohamad, Reiter, Andreas, van Anrooij, Bjorn, Gotlib, Jason, Hartmann, Karin ORCID: 0000-0002-4595-8226, Illerhaus, Anja, Elberink, Hanneke N. G. Oude, Gorska, Aleksandra, Niedoszytko, Marek, Lange, Magdalena, Scaffidi, Luigi, Zanotti, Roberta, Bonadonna, Patrizia, Perkins, Cecelia, Elena, Chiara, Malcovati, Luca, Shoumariyeh, Khalid, von Bubnoff, Nikolas, Mueller, Sabine, Triggiani, Massimo, Parente, Roberta, Schwaab, Juliana, Kundi, Michael, Fortina, Anna Belloni ORCID: 0000-0001-5791-0775, Caroppo, Francesca ORCID: 0000-0003-3583-0816, Brockow, Knut, Zink, Alexander ORCID: 0000-0001-9313-6588, Fuchs, David, Angelova-Fischer, Irena, Yavuz, Akif Selim, Doubek, Michael, Mattsson, Mattias, Hagglund, Hans, Panse, Jens, Simonowski, Anne, Sabato, Vito, Schug, Tanja, Jentzsch, Madlen ORCID: 0000-0002-2270-0804, Breynaert, Christine ORCID: 0000-0002-7368-1068, Varkonyi, Judit, Kennedy, Vanessa, Hermine, Olivier, Rossignol, Julien, Arock, Michel, Valent, Peter ORCID: 0000-0003-0456-5095 and Sperr, Wolfgang R. (2021). Cytogenetic and molecular aberrations and worse outcome for male patients in systemic mastocytosis. Theranostics, 11 (1). S. 292 - 304. LAKE HAVEN: IVYSPRING INT PUBL. ISSN 1838-7640

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Abstract

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 < 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 < 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.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Kluin-Nelemans, Hanneke C.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Jawhar, MohamadUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Reiter, AndreasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
van Anrooij, BjornUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gotlib, JasonUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hartmann, KarinUNSPECIFIEDorcid.org/0000-0002-4595-8226UNSPECIFIED
Illerhaus, AnjaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Elberink, Hanneke N. G. OudeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gorska, AleksandraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Niedoszytko, MarekUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Lange, MagdalenaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Scaffidi, LuigiUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Zanotti, RobertaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bonadonna, PatriziaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Perkins, CeceliaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Elena, ChiaraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Malcovati, LucaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Shoumariyeh, KhalidUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
von Bubnoff, NikolasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Mueller, SabineUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Triggiani, MassimoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Parente, RobertaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schwaab, JulianaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kundi, MichaelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Fortina, Anna BelloniUNSPECIFIEDorcid.org/0000-0001-5791-0775UNSPECIFIED
Caroppo, FrancescaUNSPECIFIEDorcid.org/0000-0003-3583-0816UNSPECIFIED
Brockow, KnutUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Zink, AlexanderUNSPECIFIEDorcid.org/0000-0001-9313-6588UNSPECIFIED
Fuchs, DavidUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Angelova-Fischer, IrenaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Yavuz, Akif SelimUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Doubek, MichaelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Mattsson, MattiasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hagglund, HansUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Panse, JensUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Simonowski, AnneUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Sabato, VitoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schug, TanjaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Jentzsch, MadlenUNSPECIFIEDorcid.org/0000-0002-2270-0804UNSPECIFIED
Breynaert, ChristineUNSPECIFIEDorcid.org/0000-0002-7368-1068UNSPECIFIED
Varkonyi, JuditUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kennedy, VanessaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hermine, OlivierUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Rossignol, JulienUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Arock, MichelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Valent, PeterUNSPECIFIEDorcid.org/0000-0003-0456-5095UNSPECIFIED
Sperr, Wolfgang R.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-607747
DOI: 10.7150/thno.51872
Journal or Publication Title: Theranostics
Volume: 11
Number: 1
Page Range: S. 292 - 304
Date: 2021
Publisher: IVYSPRING INT PUBL
Place of Publication: LAKE HAVEN
ISSN: 1838-7640
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
MAST-CELLS; CLONAL HEMATOPOIESIS; CLASSIFICATION; PREVALENCE; MUTATIONS; LEUKEMIA; ADULTSMultiple languages
Medicine, Research & ExperimentalMultiple languages
URI: http://kups.ub.uni-koeln.de/id/eprint/60774

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