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Verfasst von:Lübke, Johannes [VerfasserIn]   i
 Schwaab, Juliana [VerfasserIn]   i
 Christen, Deborah [VerfasserIn]   i
 Elberink, Hanneke Oude [VerfasserIn]   i
 Span, Bart [VerfasserIn]   i
 Niedoszytko, Marek [VerfasserIn]   i
 Gorska, Aleksandra [VerfasserIn]   i
 Lange, Magdalena [VerfasserIn]   i
 Gleixner, Karoline V. [VerfasserIn]   i
 Hadzijusufovic, Emir [VerfasserIn]   i
 Solomianyi, Oleksii [VerfasserIn]   i
 Angelova-Fischer, Irena [VerfasserIn]   i
 Zanotti, Roberta [VerfasserIn]   i
 Bonifacio, Massimiliano [VerfasserIn]   i
 Bonadonna, Patrizia [VerfasserIn]   i
 Shoumariyeh, Khalid [VerfasserIn]   i
 von Bubnoff, Nikolas [VerfasserIn]   i
 Müller, Sabine [VerfasserIn]   i
 Perkins, Cecelia [VerfasserIn]   i
 Elena, Chiara [VerfasserIn]   i
 Malcovati, Luca [VerfasserIn]   i
 Hagglund, Hans [VerfasserIn]   i
 Mattsson, Mattias [VerfasserIn]   i
 Parente, Roberta [VerfasserIn]   i
 Varkonyi, Judit [VerfasserIn]   i
 Fortina, Anna Belloni [VerfasserIn]   i
 Caroppo, Francesca [VerfasserIn]   i
 Zink, Alexander [VerfasserIn]   i
 Brockow, Knut [VerfasserIn]   i
 Breynaert, Christine [VerfasserIn]   i
 Bullens, Dominique [VerfasserIn]   i
 Yavuz, Akif Selim [VerfasserIn]   i
 Doubek, Michael [VerfasserIn]   i
 Sabato, Vito [VerfasserIn]   i
 Schug, Tanja [VerfasserIn]   i
 Niederwieser, Dietger [VerfasserIn]   i
 Hartmann, Karin [VerfasserIn]   i
 Triggiani, Massimo [VerfasserIn]   i
 Gotlib, Jason [VerfasserIn]   i
 Hermine, Olivier [VerfasserIn]   i
 Arock, Michel [VerfasserIn]   i
 Kluin-Nelemans, Hanneke C. [VerfasserIn]   i
 Panse, Jens [VerfasserIn]   i
 Sperr, Wolfgang R. [VerfasserIn]   i
 Valent, Peter [VerfasserIn]   i
 Reiter, Andreas [VerfasserIn]   i
 Jawhar, Mohamad [VerfasserIn]   i
Titel:Prognostic impact of organomegaly in mastocytosis
Titelzusatz:an analysis of the European Competence Network on Mastocytosis
Verf.angabe:Johannes Lübke, Juliana Schwaab, Deborah Christen, Hanneke Oude Elberink, Bart Span, Marek Niedoszytko, Aleksandra Gorska, Magdalena Lange, Karoline V. Gleixner, Emir Hadzijusufovic, Oleksii Solomianyi, Irena Angelova-Fischer, Roberta Zanotti, Massimiliano Bonifacio, Patrizia Bonadonna, Khalid Shoumariyeh, Nikolas von Bubnoff, Sabine Müller, Cecelia Perkins, Chiara Elena, Luca Malcovati, Hans Hagglund, Mattias Mattsson, Roberta Parente, Judit Varkonyi, Anna Belloni Fortina, Francesca Caroppo, Alexander Zink, Knut Brockow, Christine Breynaert, Dominique Bullens, Akif Selim Yavuz, Michael Doubek, Vito Sabato, Tanja Schug, Dietger Niederwieser, Karin Hartmann, Massimo Triggiani, Jason Gotlib, Olivier Hermine, Michel Arock, Hanneke C. Kluin-Nelemans, Jens Panse, Wolfgang R. Sperr, Peter Valent, Andreas Reiter, Mohamad Jawhar
E-Jahr:2023
Jahr:February 2023
Umfang:15 S.
Fussnoten:Online verfügbar: 17. November 2022, Artikelversion: 7. Februar 2023 ; Gesehen am 28.07.2023
Titel Quelle:Enthalten in: The journal of allergy and clinical immunology / In practice
Ort Quelle:Amsterdam [u.a.] : Elsevier, 2013
Jahr Quelle:2023
Band/Heft Quelle:11(2023), 2 vom: Feb., Seite 581-590.e5
ISSN Quelle:2213-2201
Abstract:Background - Organomegaly, including splenomegaly, hepatomegaly, and/or lymphadenopathy, are important diagnostic and prognostic features in patients with cutaneous mastocytosis (CM) or systemic mastocytosis (SM). - Objectives - To investigate the prevalence and prognostic impact of 1 or more organomegalies on clinical course and survival in patients with CM/SM. - Methods - Therefore, 3155 patients with CM (n = 1002 [32%]) or SM (n = 2153 [68%]) enrolled within the registry of the European Competence Network on Mastocytosis were analyzed. - Results - Overall survival (OS) was adversely affected by the number of organomegalies (OS: #0 vs #1 hazard ratio [HR], 4.9; 95% CI, 3.4-7.1, P < .001; #1 vs #2 HR, 2.1, 95% CI, 1.4-3.1, P < .001; #2 vs #3 HR, 1.7, 95% CI, 1.2-2.5, P = .004). Lymphadenopathy was frequently detected in patients with smoldering SM (SSM, 18 of 60 [30%]) or advanced SM (AdvSM, 137 of 344 [40%]). Its presence confered an inferior outcome in patients with AdvSM compared with patients with AdvSM without lymphadenopathy (median OS, 3.8 vs 2.6 years; HR, 1.6; 95% CI, 1.2-2.2; P = .003). OS was not different between patients having organomegaly with either ISM or SSM (median, 25.5 years vs not reached; P = .435). At time of disease progression, a new occurrence of any organomegaly was observed in 17 of 40 (43%) patients with ISM, 4 of 10 (40%) patients with SSM, and 33 of 86 (38%) patients with AdvSM, respectively. - Conclusions - Organomegalies including lymphadenopathy are often found in SSM and AdvSM. ISM with organomegaly has a similar course and prognosis compared with SSM. The number of organomegalies is adversely associated with OS. A new occurrence of organomegaly in all variants of SM may indicate disease progression.
DOI:doi:10.1016/j.jaip.2022.10.051
URL:Bitte beachten Sie: Dies ist ein Bibliographieeintrag. Ein Volltextzugriff für Mitglieder der Universität besteht hier nur, falls für die entsprechende Zeitschrift/den entsprechenden Sammelband ein Abonnement besteht oder es sich um einen OpenAccess-Titel handelt.

Volltext: https://doi.org/10.1016/j.jaip.2022.10.051
 Volltext: https://www.sciencedirect.com/science/article/pii/S2213219822011977
 DOI: https://doi.org/10.1016/j.jaip.2022.10.051
Datenträger:Online-Ressource
Sprache:eng
Sach-SW:Hepatomegaly
 Lymphadenopathy
 Mastocytosis
 Organomegaly
 Splenomegaly
 Systemic mastocytosis
K10plus-PPN:1853863602
Verknüpfungen:→ Zeitschrift

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