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Cytogenetic and mol...
Cytogenetic and molecular aberrations and worse outcome for male patients in systemic mastocytosis
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- Kluin-Nelemans, Hanneke C. (författare)
- Univ Groningen, Dept Haematol, Univ Med Ctr Groningen, Groningen, Netherlands.
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- Jawhar, Mohamad (författare)
- Heidelberg Univ, Univ Med Mannheim, Med Klin 3, Mannheim, Germany.
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- Reiter, Andreas (författare)
- Heidelberg Univ, Univ Med Mannheim, Med Klin 3, Mannheim, Germany.
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- van Anrooij, Bjorn (författare)
- Univ Groningen, Dept Haematol, Univ Med Ctr Groningen, Groningen, Netherlands.;Univ Groningen, Univ Med Ctr Groningen, Sect Allergol, Internal Med, Groningen, Netherlands.
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- Gotlib, Jason (författare)
- Stanford Univ, Dept Med, Sch Med, Div Hematol, Stanford, CA 94305 USA.
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- Hartmann, Karin (författare)
- Univ Basel, Div Allergy, Dept Dermatol, Basel, Switzerland.;Univ Basel, Dept Biomed, Basel, Switzerland.
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- Illerhaus, Anja (författare)
- Uniklin Koln, Dept Dermatol & Venerol, Cologne, Germany.
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- Elberink, Hanneke N. G. Oude (författare)
- Univ Groningen, Univ Med Ctr Groningen, Sect Allergol, Internal Med, Groningen, Netherlands.
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- Gorska, Aleksandra (författare)
- Med Univ Gdansk, Dept Allergol, Gdansk, Poland.
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- Niedoszytko, Marek (författare)
- Med Univ Gdansk, Dept Allergol, Gdansk, Poland.
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- Lange, Magdalena (författare)
- Med Univ Gdansk, Dept Dermatol Venereol & Allergol, Gdansk, Poland.
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- Scaffidi, Luigi (författare)
- Verona Univ Hosp, Dept Med, Sect Hematol, Verona, Italy.
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- Zanotti, Roberta (författare)
- Verona Univ Hosp, Dept Med, Sect Hematol, Verona, Italy.
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- Bonadonna, Patrizia (författare)
- Verona Univ Hosp, Allergy Unit, Verona, Italy.
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- Perkins, Cecelia (författare)
- Stanford Univ, Dept Med, Sch Med, Div Hematol, Stanford, CA 94305 USA.
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- Elena, Chiara (författare)
- Univ Pavia, Dept Mol Med, Pavia, Italy.;Univ Pavia, Dept Hematol Oncol, Pavia, Italy.;Fdn IRCCS Policlin San Matteo, Pavia, Italy.
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- Malcovati, Luca (författare)
- Univ Pavia, Dept Mol Med, Pavia, Italy.;Univ Pavia, Dept Hematol Oncol, Pavia, Italy.;Fdn IRCCS Policlin San Matteo, Pavia, Italy.
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- Shoumariyeh, Khalid (författare)
- Univ Freiburg, Fac Med, Med Ctr, Dept Hematol Oncol & Stem Cell Transplantat, Freiburg, Germany.;German Canc Consortium DKTK Partner Site Freiburg, Freiburg, Germany.
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- von Bubnoff, Nikolas (författare)
- Univ 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.
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- Mueller, Sabine (författare)
- German Canc Consortium DKTK Partner Site Freiburg, Freiburg, Germany.;Univ Freiburg, Med Ctr Univ Freiburg, Fac Med, Dept Dermatol, Freiburg, Germany.
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- Triggiani, Massimo (författare)
- Univ Salerno, Div Allergy & Clin Immunol, Salerno, Italy.
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- Parente, Roberta (författare)
- Univ Salerno, Div Allergy & Clin Immunol, Salerno, Italy.
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- Schwaab, Juliana (författare)
- Heidelberg Univ, Univ Med Mannheim, Med Klin 3, Mannheim, Germany.
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- Kundi, Michael (författare)
- Med Univ Vienna, Inst Environm Hlth, Vienna, Austria.
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- Fortina, Anna Belloni (författare)
- Univ Padua, Dept Med, Pediat Dermatol Unit, Padua, Italy.
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- Caroppo, Francesca (författare)
- Univ Padua, Dept Med, Pediat Dermatol Unit, Padua, Italy.
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- Brockow, Knut (författare)
- Tech Univ Munich, Sch Med, Dept Dermatol & Allergy Biederstein, Munich, Germany.
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- Zink, Alexander (författare)
- Tech Univ Munich, Sch Med, Dept Dermatol & Allergy Biederstein, Munich, Germany.
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- Fuchs, David (författare)
- Johannes Kepler Univ Linz, Univ Clin Hematol & Oncol, Kepler Univ Hosp, Linz, Austria.
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- Angelova-Fischer, Irena (författare)
- Kepler Univ Hosp, Dept Dermatol & Venereol, Allergy Ctr, Linz, Austria.
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- Yavuz, Akif Selim (författare)
- Istanbul Univ, Istanbul Med Sch, Div Hematol, Istanbul, Turkey.
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- Doubek, Michael (författare)
- Univ Hosp, Brno, Czech Republic.
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- Mattsson, Mattias (författare)
- Uppsala universitet,Experimentell och klinisk onkologi,Hematologi
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- Hägglund, Hans (författare)
- Uppsala universitet,Hematologi
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- Panse, Jens (författare)
- Univ Hosp RWTH Aachen, Dept Oncol Hematol Hemostaseol & Stem Cell Transp, Aachen, Germany.
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- Simonowski, Anne (författare)
- Univ Hosp RWTH Aachen, Dept Oncol Hematol Hemostaseol & Stem Cell Transp, Aachen, Germany.
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- Sabato, Vito (författare)
- Univ Antwerp, Fac Med & Hlth Sci, Dept Immunol Allergol Rheumatol, Antwerp, Belgium.
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- Schug, Tanja (författare)
- Med Univ Graz, Dept Dermatol & Venereol, Graz, Austria.
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- Jentzsch, Madlen (författare)
- Leipzig Univ Hosp, Hematol & Cellular Therapy, Med Clin & Policlin 1, Leipzig, Germany.
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- Breynaert, Christine (författare)
- Univ Hosp Leuven, Dept Dermatol, Leuven, Belgium.
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- Varkonyi, Judit (författare)
- Semmelweis Univ, Dept Hematol, Budapest, Hungary.
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- Kennedy, Vanessa (författare)
- Univ Calif San Francisco, Dept Med, San Francisco, CA 94143 USA.
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- Hermine, Olivier (författare)
- Univ Paris 05, French Reference Ctr Mastocytosis CEREMAST, Imagine Inst, Hop Necker,Assistance Publ Hop Paris, Paris, France.
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- Rossignol, Julien (författare)
- Gustave Roussy Canc Ctr, Dept Hematol, Villejuif, France.
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- Arock, Michel (författare)
- Piti Salpetriere Hosp, Lab Hematol, Paris, France.
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- Valent, Peter (författare)
- Med Univ Vienna, Dept Internal Med 1, Div Hematol & Hemostaseol, Vienna, Austria.;Ludwig Boltzmann Inst Hematol & Oncol, Vienna, Austria.
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- Sperr, Wolfgang R. (författare)
- Med Univ Vienna, Dept Internal Med 1, Div Hematol & Hemostaseol, Vienna, Austria.;Ludwig Boltzmann Inst Hematol & Oncol, Vienna, Austria.
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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)
- Ivyspring International Publisher, 2021
- 2021
- Engelska.
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Ingår i: Theranostics. - : Ivyspring International Publisher. - 1838-7640. ; 11:1, s. 292-303
- Relaterad länk:
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https://doi.org/10.7...
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https://uu.diva-port... (primary) (Raw object)
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https://doi.org/10.7...
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https://urn.kb.se/re...
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https://doi.org/10.7...
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Abstract
Ämnesord
Stäng
- 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.
Ämnesord
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Hematologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Hematology (hsv//eng)
Nyckelord
- Mastocytosis
- sex difference
- cytogenetics
- molecular mutations
- survival
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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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visa fler...
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Illerhaus, Anja
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Elberink, Hannek ...
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Gorska, Aleksand ...
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Niedoszytko, Mar ...
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Lange, Magdalena
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Scaffidi, Luigi
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Zanotti, Roberta
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Bonadonna, Patri ...
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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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Triggiani, Massi ...
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Parente, Roberta
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Schwaab, Juliana
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Kundi, Michael
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Fortina, Anna Be ...
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Caroppo, Frances ...
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Brockow, Knut
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Zink, Alexander
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Fuchs, David
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Angelova-Fischer ...
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Yavuz, Akif Seli ...
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Doubek, Michael
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Mattsson, Mattia ...
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Hägglund, Hans
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Panse, Jens
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Simonowski, Anne
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Sabato, Vito
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Schug, Tanja
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Jentzsch, Madlen
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Breynaert, Chris ...
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Varkonyi, Judit
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Kennedy, Vanessa
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Hermine, Olivier
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Rossignol, Julie ...
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Arock, Michel
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Valent, Peter
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Sperr, Wolfgang ...
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