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Prognostic Impact o...
Prognostic Impact of Organomegaly in Mastocytosis : An Analysis of the European Competence Network on Mastocytosis
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- Lübke, Johannes (författare)
- Heidelberg Univ, Univ Hosp Mannheim, Dept Hematol & Oncol, Mannheim, Germany.
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- Schwaab, Juliana (författare)
- Heidelberg Univ, Univ Hosp Mannheim, Dept Hematol & Oncol, Mannheim, Germany.
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- Christen, Deborah (författare)
- Univ Hosp RWTH Aachen, Dept Oncol Haematol Haemostaseol & Stem Cell Tran, Aachen, Germany.
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- Elberink, Hanneke Oude (författare)
- Univ Groningen, Univ Med Ctr Groningen, Dept Allergol, Groningen, Netherlands.
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- Span, Bart (författare)
- Univ Groningen, Univ Med Ctr Groningen, Dept Hematol, Groningen, Netherlands.
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- Niedoszytko, Marek (författare)
- Med Univ Gdansk, Dept Allergol, Gdansk, Poland.
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- Gorska, Aleksandra (författare)
- Med Univ Gdansk, Dept Allergol, Gdansk, Poland.
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- Lange, Magdalena (författare)
- Med Univ Gdansk, Dept Dermatol, Gdansk, Poland.
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- Gleixner, Karoline V. (författare)
- Med Univ Vienna, Dept Int Med 1, Div Hematol & Hemostaseol, Vienna, Austria.;Med Univ Vienna, Ludwig Boltzmann Inst Hematol & Oncol, Vienna, Austria.
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- Hadzijusufovic, Emir (författare)
- Med 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.
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- Solomianyi, Oleksii (författare)
- Johannes Kepler Univ Linz, Univ Clin Hematol & Oncol, Kepler Univ Hosp, Linz, Austria.
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- Angelova-Fischer, Irena (författare)
- Johannes Kepler Univ Linz, Kepler Univ Hosp, Allergy Ctr, Dept Dermatol & Venereol, Linz, Austria.
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- Zanotti, Roberta (författare)
- Verona Univ Hosp, Dept Med, Sect Hematol, Verona, Italy.
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- Bonifacio, Massimiliano (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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- Shoumariyeh, Khalid (författare)
- Univ Freiburg, Fac Med, Med Ctr, Dept Hematol & Oncol, 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, 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)
- Univ Freiburg, Fac Med, Med Ctr, Dept Dermatol, Freiburg, Germany.
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- Perkins, Cecelia (författare)
- Stanford Univ, Sch Med, Dept 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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- Hägglund, Hans (författare)
- Uppsala universitet,Hematologi,Uppsala Univ, Dept Med Sci, Div Hematol, Uppsala, Sweden.
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- Mattsson, Mattias (författare)
- Uppsala universitet,Hematologi
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- Parente, Roberta (författare)
- Univ Salerno, Div Allergy & Clin Immunol, Salerno, Italy.
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- Varkonyi, Judit (författare)
- Semmelwe Univ, Dept Hematol, Budapest, Hungary.
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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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- Zink, Alexander (författare)
- Karolinska Institutet,Techn Univ Munchen, Fac Med, Dept Dermatol & Allergy Biederstein, Munich, Germany.
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- Brockow, Knut (författare)
- Techn Univ Munchen, Fac Med, Dept Dermatol & Allergy Biederstein, Munich, Germany.
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- Breynaert, Christine (författare)
- Katholieke Univ Leuven, Dept Microbiol Immunol & Transplantat, Allergy & Clin Immunol Res Grp, Leuven, Belgium.;Univ Hosp Leuven, MASTeL, Leuven, Belgium.
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- Bullens, Dominique (författare)
- Katholieke Univ Leuven, Dept Microbiol Immunol & Transplantat, Allergy & Clin Immunol Res Grp, Leuven, Belgium.;Univ Hosp Leuven, MASTeL, Leuven, Belgium.
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- Yavuz, Akif Selim (författare)
- Istanbul Univ, Istanbul Med Sch, Div Hematol, Istanbul, Turkiye.
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- Doubek, Michael (författare)
- Univ Hosp & Fac Med, Brno, Czech Republic.
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- Sabato, Vito (författare)
- Univ Antwerp, Fac Med & Hlth Sci, Dept Immunol Allergol Rheumatol, Antwerp, Belgium.;Antwerp Univ Hosp, Antwerp, Belgium.
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- Schug, Tanja (författare)
- Univ Hosp Graz, Dept Dermatol & Venereol, Graz, Austria.
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- Niederwieser, Dietger (författare)
- Univ Hosp Leipzig, Leipzig, Germany.
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- Hartmann, Karin (författare)
- Univ Basel, Dept Dermatol, Div Allergy, Basel, Switzerland.;Uni Hosp Basel, Dept Biomed, Basel, Switzerland.;Univ Basel, Basel, Switzerland.
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- Triggiani, Massimo (författare)
- Univ Salerno, Div Allergy & Clin Immunol, Salerno, Italy.
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- Gotlib, Jason (författare)
- Stanford Univ, Sch Med, Dept Med, Div Hematol, Stanford, CA 94305 USA.
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- Hermine, Olivier (författare)
- Univ Paris 05, Hop Necker, AP HP, Imagine Inst,French Reference Ctr Mastocytosis CE, Paris, France.
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- Arock, Michel (författare)
- Hop La Pitie Salpetriere, Lab Hematol, Paris, France.
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- Kluin-Nelemans, Hanneke C. (författare)
- Univ Groningen, Univ Med Ctr Groningen, Dept Hematol, Groningen, Netherlands.
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- Panse, Jens (författare)
- Univ Hosp RWTH Aachen, Dept Oncol Haematol Haemostaseol & Stem Cell Tran, Aachen, Germany.;Aachen Bonn Cologne Dusseldorf CIO ABCD, Ctr Integrated Oncol, Aachen, Germany.
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- Sperr, Wolfgang R. (författare)
- Med Univ Vienna, Dept Int Med 1, Div Hematol & Hemostaseol, Vienna, Austria.;Med Univ Vienna, Ludwig Boltzmann Inst Hematol & Oncol, Vienna, Austria.
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Valent, Peter (författare)
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- Reiter, Andreas (författare)
- Heidelberg Univ, Univ Hosp Mannheim, Dept Hematol & Oncol, Mannheim, Germany.
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- Jawhar, Mohamad (författare)
- Heidelberg Univ, Univ Hosp Mannheim, Dept Hematol & Oncol, Mannheim, Germany.
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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)
- Elsevier, 2023
- 2023
- Engelska.
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Ingår i: Journal of Allergy and Clinical Immunology. - : Elsevier. - 2213-2198 .- 2213-2201. ; 11:2, s. 581-590
- Relaterad länk:
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https://urn.kb.se/re...
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https://doi.org/10.1...
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Abstract
Ämnesord
Stäng
- 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
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Hematologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Hematology (hsv//eng)
Nyckelord
- Mastocytosis
- Systemic mastocytosis
- Organomegaly
- Splenomegaly
- Hepatomegaly
- Lymphadenopathy
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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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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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