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Cytogenetic and mol...
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Kluin-Nelemans, Hanneke C.Univ Groningen, Dept Haematol, Univ Med Ctr Groningen, Groningen, Netherlands.
(author)
Cytogenetic and molecular aberrations and worse outcome for male patients in systemic mastocytosis
- Article/chapterEnglish2021
Publisher, publication year, extent ...
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Ivyspring International Publisher,2021
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electronicrdacarrier
Numbers
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LIBRIS-ID:oai:DiVA.org:uu-425895
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https://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-425895URI
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https://doi.org/10.7150/thno.51872DOI
Supplementary language notes
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Language:English
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Summary in:English
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Classification
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Subject category:ref swepub-contenttype
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Subject category:art swepub-publicationtype
Notes
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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.
Subject headings and genre
Added entries (persons, corporate bodies, meetings, titles ...)
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Jawhar, MohamadHeidelberg Univ, Univ Med Mannheim, Med Klin 3, Mannheim, Germany.
(author)
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Reiter, AndreasHeidelberg Univ, Univ Med Mannheim, Med Klin 3, Mannheim, Germany.
(author)
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van Anrooij, BjornUniv Groningen, Dept Haematol, Univ Med Ctr Groningen, Groningen, Netherlands.;Univ Groningen, Univ Med Ctr Groningen, Sect Allergol, Internal Med, Groningen, Netherlands.
(author)
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Gotlib, JasonStanford Univ, Dept Med, Sch Med, Div Hematol, Stanford, CA 94305 USA.
(author)
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Hartmann, KarinUniv Basel, Div Allergy, Dept Dermatol, Basel, Switzerland.;Univ Basel, Dept Biomed, Basel, Switzerland.
(author)
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Illerhaus, AnjaUniklin Koln, Dept Dermatol & Venerol, Cologne, Germany.
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Elberink, Hanneke N. G. OudeUniv Groningen, Univ Med Ctr Groningen, Sect Allergol, Internal Med, Groningen, Netherlands.
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Gorska, AleksandraMed Univ Gdansk, Dept Allergol, Gdansk, Poland.
(author)
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Niedoszytko, MarekMed Univ Gdansk, Dept Allergol, Gdansk, Poland.
(author)
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Lange, MagdalenaMed Univ Gdansk, Dept Dermatol Venereol & Allergol, Gdansk, Poland.
(author)
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Scaffidi, LuigiVerona Univ Hosp, Dept Med, Sect Hematol, Verona, Italy.
(author)
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Zanotti, RobertaVerona Univ Hosp, Dept Med, Sect Hematol, Verona, Italy.
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Bonadonna, PatriziaVerona Univ Hosp, Allergy Unit, Verona, Italy.
(author)
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Perkins, CeceliaStanford Univ, Dept Med, Sch Med, Div Hematol, Stanford, CA 94305 USA.
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Elena, ChiaraUniv Pavia, Dept Mol Med, Pavia, Italy.;Univ Pavia, Dept Hematol Oncol, Pavia, Italy.;Fdn IRCCS Policlin San Matteo, Pavia, Italy.
(author)
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Malcovati, LucaUniv Pavia, Dept Mol Med, Pavia, Italy.;Univ Pavia, Dept Hematol Oncol, Pavia, Italy.;Fdn IRCCS Policlin San Matteo, Pavia, Italy.
(author)
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Shoumariyeh, KhalidUniv Freiburg, Fac Med, Med Ctr, Dept Hematol Oncol & Stem Cell Transplantat, Freiburg, Germany.;German Canc Consortium DKTK Partner Site Freiburg, Freiburg, Germany.
(author)
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von Bubnoff, NikolasUniv Freiburg, Fac Med, Med Ctr, Dept Hematol Oncol & Stem Cell Transplantat, Freiburg, Germany.;German Canc Consortium DKTK Partner Site Freiburg, Freiburg, Germany.;Univ Schleswig Holstein, Med Ctr, Dept Hematol & Oncol, Campus Lubeck, Lubeck, Germany.
(author)
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Mueller, SabineGerman Canc Consortium DKTK Partner Site Freiburg, Freiburg, Germany.;Univ Freiburg, Med Ctr Univ Freiburg, Fac Med, Dept Dermatol, Freiburg, Germany.
(author)
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Triggiani, MassimoUniv Salerno, Div Allergy & Clin Immunol, Salerno, Italy.
(author)
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Parente, RobertaUniv Salerno, Div Allergy & Clin Immunol, Salerno, Italy.
(author)
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Schwaab, JulianaHeidelberg Univ, Univ Med Mannheim, Med Klin 3, Mannheim, Germany.
(author)
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Kundi, MichaelMed Univ Vienna, Inst Environm Hlth, Vienna, Austria.
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Fortina, Anna BelloniUniv Padua, Dept Med, Pediat Dermatol Unit, Padua, Italy.
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Caroppo, FrancescaUniv Padua, Dept Med, Pediat Dermatol Unit, Padua, Italy.
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Brockow, KnutTech Univ Munich, Sch Med, Dept Dermatol & Allergy Biederstein, Munich, Germany.
(author)
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Zink, AlexanderTech Univ Munich, Sch Med, Dept Dermatol & Allergy Biederstein, Munich, Germany.
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Fuchs, DavidJohannes Kepler Univ Linz, Univ Clin Hematol & Oncol, Kepler Univ Hosp, Linz, Austria.
(author)
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Angelova-Fischer, IrenaKepler Univ Hosp, Dept Dermatol & Venereol, Allergy Ctr, Linz, Austria.
(author)
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Yavuz, Akif SelimIstanbul Univ, Istanbul Med Sch, Div Hematol, Istanbul, Turkey.
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Doubek, MichaelUniv Hosp, Brno, Czech Republic.
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Mattsson, MattiasUppsala universitet,Experimentell och klinisk onkologi,Hematologi(Swepub:uu)matma431
(author)
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Hägglund, HansUppsala universitet,Hematologi(Swepub:uu)hanha689
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Panse, JensUniv Hosp RWTH Aachen, Dept Oncol Hematol Hemostaseol & Stem Cell Transp, Aachen, Germany.
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Simonowski, AnneUniv Hosp RWTH Aachen, Dept Oncol Hematol Hemostaseol & Stem Cell Transp, Aachen, Germany.
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Sabato, VitoUniv Antwerp, Fac Med & Hlth Sci, Dept Immunol Allergol Rheumatol, Antwerp, Belgium.
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Schug, TanjaMed Univ Graz, Dept Dermatol & Venereol, Graz, Austria.
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Jentzsch, MadlenLeipzig Univ Hosp, Hematol & Cellular Therapy, Med Clin & Policlin 1, Leipzig, Germany.
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Breynaert, ChristineUniv Hosp Leuven, Dept Dermatol, Leuven, Belgium.
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Varkonyi, JuditSemmelweis Univ, Dept Hematol, Budapest, Hungary.
(author)
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Kennedy, VanessaUniv Calif San Francisco, Dept Med, San Francisco, CA 94143 USA.
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Hermine, OlivierUniv Paris 05, French Reference Ctr Mastocytosis CEREMAST, Imagine Inst, Hop Necker,Assistance Publ Hop Paris, Paris, France.
(author)
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Rossignol, JulienGustave Roussy Canc Ctr, Dept Hematol, Villejuif, France.
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Arock, MichelPiti Salpetriere Hosp, Lab Hematol, Paris, France.
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Valent, PeterMed Univ Vienna, Dept Internal Med 1, Div Hematol & Hemostaseol, Vienna, Austria.;Ludwig Boltzmann Inst Hematol & Oncol, Vienna, Austria.
(author)
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Sperr, Wolfgang R.Med Univ Vienna, Dept Internal Med 1, Div Hematol & Hemostaseol, Vienna, Austria.;Ludwig Boltzmann Inst Hematol & Oncol, Vienna, Austria.
(author)
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Univ Groningen, Dept Haematol, Univ Med Ctr Groningen, Groningen, Netherlands.Heidelberg Univ, Univ Med Mannheim, Med Klin 3, Mannheim, Germany.
(creator_code:org_t)
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In:Theranostics: Ivyspring International Publisher11:1, s. 292-3031838-7640
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Kluin-Nelemans, ...
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Jawhar, Mohamad
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Reiter, Andreas
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van Anrooij, Bjo ...
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Gotlib, Jason
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Hartmann, Karin
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Illerhaus, Anja
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Elberink, Hannek ...
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Niedoszytko, Mar ...
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Scaffidi, Luigi
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Zanotti, Roberta
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Perkins, Cecelia
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Elena, Chiara
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Malcovati, Luca
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Shoumariyeh, Kha ...
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von Bubnoff, Nik ...
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Mueller, Sabine
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Schwaab, Juliana
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Brockow, Knut
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Zink, Alexander
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Doubek, Michael
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