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Prognostic Impact o...
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Lübke, JohannesHeidelberg Univ, Univ Hosp Mannheim, Dept Hematol & Oncol, Mannheim, Germany.
(författare)
Prognostic Impact of Organomegaly in Mastocytosis : An Analysis of the European Competence Network on Mastocytosis
- Artikel/kapitelEngelska2023
Förlag, utgivningsår, omfång ...
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Elsevier,2023
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printrdacarrier
Nummerbeteckningar
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LIBRIS-ID:oai:DiVA.org:uu-507040
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https://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-507040URI
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https://doi.org/10.1016/j.jaip.2022.10.051DOI
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http://kipublications.ki.se/Default.aspx?queryparsed=id:152841587URI
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Språk:engelska
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Sammanfattning på:engelska
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Ämneskategori:ref swepub-contenttype
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Ämneskategori:art swepub-publicationtype
Anmärkningar
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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.
Ämnesord och genrebeteckningar
Biuppslag (personer, institutioner, konferenser, titlar ...)
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Schwaab, JulianaHeidelberg Univ, Univ Hosp Mannheim, Dept Hematol & Oncol, Mannheim, Germany.
(författare)
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Christen, DeborahUniv Hosp RWTH Aachen, Dept Oncol Haematol Haemostaseol & Stem Cell Tran, Aachen, Germany.
(författare)
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Elberink, Hanneke OudeUniv Groningen, Univ Med Ctr Groningen, Dept Allergol, Groningen, Netherlands.
(författare)
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Span, BartUniv Groningen, Univ Med Ctr Groningen, Dept Hematol, Groningen, Netherlands.
(författare)
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Niedoszytko, MarekMed Univ Gdansk, Dept Allergol, Gdansk, Poland.
(författare)
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Gorska, AleksandraMed Univ Gdansk, Dept Allergol, Gdansk, Poland.
(författare)
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Lange, MagdalenaMed Univ Gdansk, Dept Dermatol, Gdansk, Poland.
(författare)
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Gleixner, Karoline V.Med Univ Vienna, Dept Int Med 1, Div Hematol & Hemostaseol, Vienna, Austria.;Med Univ Vienna, Ludwig Boltzmann Inst Hematol & Oncol, Vienna, Austria.
(författare)
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Hadzijusufovic, EmirMed Univ Vienna, Dept Int Med 1, Div Hematol & Hemostaseol, Vienna, Austria.;Med Univ Vienna, Ludwig Boltzmann Inst Hematol & Oncol, Vienna, Austria.;Univ Vet Med, Univ Clin Small Anim, Dept Univ Clin Compan Anim & Horses, Internal Med Small Anim, Vienna, Austria.
(författare)
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Solomianyi, OleksiiJohannes Kepler Univ Linz, Univ Clin Hematol & Oncol, Kepler Univ Hosp, Linz, Austria.
(författare)
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Angelova-Fischer, IrenaJohannes Kepler Univ Linz, Kepler Univ Hosp, Allergy Ctr, Dept Dermatol & Venereol, Linz, Austria.
(författare)
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Zanotti, RobertaVerona Univ Hosp, Dept Med, Sect Hematol, Verona, Italy.
(författare)
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Bonifacio, MassimilianoVerona Univ Hosp, Dept Med, Sect Hematol, Verona, Italy.
(författare)
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Bonadonna, PatriziaVerona Univ Hosp, Allergy Unit, Verona, Italy.
(författare)
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Shoumariyeh, KhalidUniv Freiburg, Fac Med, Med Ctr, Dept Hematol & Oncol, Freiburg, Germany.;German Canc Consortium DKTK Partner Site Freiburg, Freiburg, Germany.
(författare)
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von Bubnoff, NikolasUniv Freiburg, Fac Med, Med Ctr, Dept Hematol & Oncol, Freiburg, Germany.;German Canc Consortium DKTK Partner Site Freiburg, Freiburg, Germany.;Univ Schleswig Holstein, Med Ctr, Dept Hematol & Oncol, Campus Lubeck, Lubeck, Germany.
(författare)
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Mueller, SabineUniv Freiburg, Fac Med, Med Ctr, Dept Dermatol, Freiburg, Germany.
(författare)
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Perkins, CeceliaStanford Univ, Sch Med, Dept Med, Div Hematol, Stanford, CA 94305 USA.
(författare)
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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.
(författare)
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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.
(författare)
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Hägglund, HansUppsala universitet,Hematologi,Uppsala Univ, Dept Med Sci, Div Hematol, Uppsala, Sweden.(Swepub:uu)hanha689
(författare)
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Mattsson, MattiasUppsala universitet,Hematologi(Swepub:uu)matma431
(författare)
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Parente, RobertaUniv Salerno, Div Allergy & Clin Immunol, Salerno, Italy.
(författare)
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Varkonyi, JuditSemmelwe Univ, Dept Hematol, Budapest, Hungary.
(författare)
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Fortina, Anna BelloniUniv Padua, Dept Med, Pediat Dermatol Unit, Padua, Italy.
(författare)
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Caroppo, FrancescaUniv Padua, Dept Med, Pediat Dermatol Unit, Padua, Italy.
(författare)
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Zink, AlexanderKarolinska Institutet,Techn Univ Munchen, Fac Med, Dept Dermatol & Allergy Biederstein, Munich, Germany.
(författare)
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Brockow, KnutTechn Univ Munchen, Fac Med, Dept Dermatol & Allergy Biederstein, Munich, Germany.
(författare)
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Breynaert, ChristineKatholieke Univ Leuven, Dept Microbiol Immunol & Transplantat, Allergy & Clin Immunol Res Grp, Leuven, Belgium.;Univ Hosp Leuven, MASTeL, Leuven, Belgium.
(författare)
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Bullens, DominiqueKatholieke Univ Leuven, Dept Microbiol Immunol & Transplantat, Allergy & Clin Immunol Res Grp, Leuven, Belgium.;Univ Hosp Leuven, MASTeL, Leuven, Belgium.
(författare)
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Yavuz, Akif SelimIstanbul Univ, Istanbul Med Sch, Div Hematol, Istanbul, Turkiye.
(författare)
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Doubek, MichaelUniv Hosp & Fac Med, Brno, Czech Republic.
(författare)
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Sabato, VitoUniv Antwerp, Fac Med & Hlth Sci, Dept Immunol Allergol Rheumatol, Antwerp, Belgium.;Antwerp Univ Hosp, Antwerp, Belgium.
(författare)
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Schug, TanjaUniv Hosp Graz, Dept Dermatol & Venereol, Graz, Austria.
(författare)
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Niederwieser, DietgerUniv Hosp Leipzig, Leipzig, Germany.
(författare)
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Hartmann, KarinUniv Basel, Dept Dermatol, Div Allergy, Basel, Switzerland.;Uni Hosp Basel, Dept Biomed, Basel, Switzerland.;Univ Basel, Basel, Switzerland.
(författare)
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Triggiani, MassimoUniv Salerno, Div Allergy & Clin Immunol, Salerno, Italy.
(författare)
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Gotlib, JasonStanford Univ, Sch Med, Dept Med, Div Hematol, Stanford, CA 94305 USA.
(författare)
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Hermine, OlivierUniv Paris 05, Hop Necker, AP HP, Imagine Inst,French Reference Ctr Mastocytosis CE, Paris, France.
(författare)
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Arock, MichelHop La Pitie Salpetriere, Lab Hematol, Paris, France.
(författare)
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Kluin-Nelemans, Hanneke C.Univ Groningen, Univ Med Ctr Groningen, Dept Hematol, Groningen, Netherlands.
(författare)
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Panse, JensUniv Hosp RWTH Aachen, Dept Oncol Haematol Haemostaseol & Stem Cell Tran, Aachen, Germany.;Aachen Bonn Cologne Dusseldorf CIO ABCD, Ctr Integrated Oncol, Aachen, Germany.
(författare)
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Sperr, Wolfgang R.Med Univ Vienna, Dept Int Med 1, Div Hematol & Hemostaseol, Vienna, Austria.;Med Univ Vienna, Ludwig Boltzmann Inst Hematol & Oncol, Vienna, Austria.
(författare)
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Valent, Peter
(författare)
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Reiter, AndreasHeidelberg Univ, Univ Hosp Mannheim, Dept Hematol & Oncol, Mannheim, Germany.
(författare)
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Jawhar, MohamadHeidelberg Univ, Univ Hosp Mannheim, Dept Hematol & Oncol, Mannheim, Germany.
(författare)
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Heidelberg Univ, Univ Hosp Mannheim, Dept Hematol & Oncol, Mannheim, Germany.Univ Hosp RWTH Aachen, Dept Oncol Haematol Haemostaseol & Stem Cell Tran, Aachen, Germany.
(creator_code:org_t)
Sammanhörande titlar
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Ingår i:Journal of Allergy and Clinical Immunology: Elsevier11:2, s. 581-5902213-21982213-2201
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Lübke, Johannes
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Schwaab, Juliana
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Christen, Debora ...
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Elberink, Hannek ...
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Span, Bart
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Niedoszytko, Mar ...
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visa fler...
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Gorska, Aleksand ...
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Lange, Magdalena
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Gleixner, Karoli ...
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Hadzijusufovic, ...
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Solomianyi, Olek ...
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Angelova-Fischer ...
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Zanotti, Roberta
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Bonifacio, Massi ...
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Bonadonna, Patri ...
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Shoumariyeh, Kha ...
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von Bubnoff, Nik ...
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Mueller, Sabine
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Perkins, Cecelia
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Elena, Chiara
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Malcovati, Luca
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Hägglund, Hans
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Mattsson, Mattia ...
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Parente, Roberta
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Varkonyi, Judit
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Fortina, Anna Be ...
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Caroppo, Frances ...
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Zink, Alexander
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Brockow, Knut
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Breynaert, Chris ...
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Bullens, Dominiq ...
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Yavuz, Akif Seli ...
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Doubek, Michael
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Sabato, Vito
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Schug, Tanja
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Niederwieser, Di ...
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Hartmann, Karin
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Triggiani, Massi ...
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Gotlib, Jason
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Hermine, Olivier
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Arock, Michel
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Kluin-Nelemans, ...
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Panse, Jens
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Sperr, Wolfgang ...
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Valent, Peter
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Reiter, Andreas
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Jawhar, Mohamad
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