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FältnamnIndikatorerMetadata
00009600naa a2200937 4500
001oai:DiVA.org:uu-507040
003SwePub
008230704s2023 | |||||||||||000 ||eng|
009oai:prod.swepub.kib.ki.se:152841587
0247 a https://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-5070402 URI
0247 a https://doi.org/10.1016/j.jaip.2022.10.0512 DOI
0247 a http://kipublications.ki.se/Default.aspx?queryparsed=id:1528415872 URI
040 a (SwePub)uud (SwePub)ki
041 a engb eng
042 9 SwePub
072 7a ref2 swepub-contenttype
072 7a art2 swepub-publicationtype
1001 a Lübke, Johannesu Heidelberg Univ, Univ Hosp Mannheim, Dept Hematol & Oncol, Mannheim, Germany.4 aut
2451 0a Prognostic Impact of Organomegaly in Mastocytosis :b An Analysis of the European Competence Network on Mastocytosis
264 1b Elsevier,c 2023
338 a print2 rdacarrier
520 a 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.
650 7a MEDICIN OCH HÄLSOVETENSKAPx Klinisk medicinx Hematologi0 (SwePub)302022 hsv//swe
650 7a MEDICAL AND HEALTH SCIENCESx Clinical Medicinex Hematology0 (SwePub)302022 hsv//eng
653 a Mastocytosis
653 a Systemic mastocytosis
653 a Organomegaly
653 a Splenomegaly
653 a Hepatomegaly
653 a Lymphadenopathy
7001 a Schwaab, Julianau Heidelberg Univ, Univ Hosp Mannheim, Dept Hematol & Oncol, Mannheim, Germany.4 aut
7001 a Christen, Deborahu Univ Hosp RWTH Aachen, Dept Oncol Haematol Haemostaseol & Stem Cell Tran, Aachen, Germany.4 aut
7001 a Elberink, Hanneke Oudeu Univ Groningen, Univ Med Ctr Groningen, Dept Allergol, Groningen, Netherlands.4 aut
7001 a Span, Bartu Univ Groningen, Univ Med Ctr Groningen, Dept Hematol, Groningen, Netherlands.4 aut
7001 a Niedoszytko, Mareku Med Univ Gdansk, Dept Allergol, Gdansk, Poland.4 aut
7001 a Gorska, Aleksandrau Med Univ Gdansk, Dept Allergol, Gdansk, Poland.4 aut
7001 a Lange, Magdalenau Med Univ Gdansk, Dept Dermatol, Gdansk, Poland.4 aut
7001 a Gleixner, Karoline V.u Med Univ Vienna, Dept Int Med 1, Div Hematol & Hemostaseol, Vienna, Austria.;Med Univ Vienna, Ludwig Boltzmann Inst Hematol & Oncol, Vienna, Austria.4 aut
7001 a Hadzijusufovic, Emiru 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.4 aut
7001 a Solomianyi, Oleksiiu Johannes Kepler Univ Linz, Univ Clin Hematol & Oncol, Kepler Univ Hosp, Linz, Austria.4 aut
7001 a Angelova-Fischer, Irenau Johannes Kepler Univ Linz, Kepler Univ Hosp, Allergy Ctr, Dept Dermatol & Venereol, Linz, Austria.4 aut
7001 a Zanotti, Robertau Verona Univ Hosp, Dept Med, Sect Hematol, Verona, Italy.4 aut
7001 a Bonifacio, Massimilianou Verona Univ Hosp, Dept Med, Sect Hematol, Verona, Italy.4 aut
7001 a Bonadonna, Patriziau Verona Univ Hosp, Allergy Unit, Verona, Italy.4 aut
7001 a Shoumariyeh, Khalidu Univ Freiburg, Fac Med, Med Ctr, Dept Hematol & Oncol, Freiburg, Germany.;German Canc Consortium DKTK Partner Site Freiburg, Freiburg, Germany.4 aut
7001 a von Bubnoff, Nikolasu 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.4 aut
7001 a Mueller, Sabineu Univ Freiburg, Fac Med, Med Ctr, Dept Dermatol, Freiburg, Germany.4 aut
7001 a Perkins, Ceceliau Stanford Univ, Sch Med, Dept Med, Div Hematol, Stanford, CA 94305 USA.4 aut
7001 a Elena, Chiarau Univ Pavia, Dept Mol Med, Pavia, Italy.;Univ Pavia, Dept Hematol Oncol, Pavia, Italy.;Fdn IRCCS Policlin San Matteo, Pavia, Italy.4 aut
7001 a Malcovati, Lucau Univ Pavia, Dept Mol Med, Pavia, Italy.;Univ Pavia, Dept Hematol Oncol, Pavia, Italy.;Fdn IRCCS Policlin San Matteo, Pavia, Italy.4 aut
7001 a Hägglund, Hansu Uppsala universitet,Hematologi,Uppsala Univ, Dept Med Sci, Div Hematol, Uppsala, Sweden.4 aut0 (Swepub:uu)hanha689
7001 a Mattsson, Mattiasu Uppsala universitet,Hematologi4 aut0 (Swepub:uu)matma431
7001 a Parente, Robertau Univ Salerno, Div Allergy & Clin Immunol, Salerno, Italy.4 aut
7001 a Varkonyi, Juditu Semmelwe Univ, Dept Hematol, Budapest, Hungary.4 aut
7001 a Fortina, Anna Belloniu Univ Padua, Dept Med, Pediat Dermatol Unit, Padua, Italy.4 aut
7001 a Caroppo, Francescau Univ Padua, Dept Med, Pediat Dermatol Unit, Padua, Italy.4 aut
7001 a Zink, Alexanderu Karolinska Institutet,Techn Univ Munchen, Fac Med, Dept Dermatol & Allergy Biederstein, Munich, Germany.4 aut
7001 a Brockow, Knutu Techn Univ Munchen, Fac Med, Dept Dermatol & Allergy Biederstein, Munich, Germany.4 aut
7001 a Breynaert, Christineu Katholieke Univ Leuven, Dept Microbiol Immunol & Transplantat, Allergy & Clin Immunol Res Grp, Leuven, Belgium.;Univ Hosp Leuven, MASTeL, Leuven, Belgium.4 aut
7001 a Bullens, Dominiqueu Katholieke Univ Leuven, Dept Microbiol Immunol & Transplantat, Allergy & Clin Immunol Res Grp, Leuven, Belgium.;Univ Hosp Leuven, MASTeL, Leuven, Belgium.4 aut
7001 a Yavuz, Akif Selimu Istanbul Univ, Istanbul Med Sch, Div Hematol, Istanbul, Turkiye.4 aut
7001 a Doubek, Michaelu Univ Hosp & Fac Med, Brno, Czech Republic.4 aut
7001 a Sabato, Vitou Univ Antwerp, Fac Med & Hlth Sci, Dept Immunol Allergol Rheumatol, Antwerp, Belgium.;Antwerp Univ Hosp, Antwerp, Belgium.4 aut
7001 a Schug, Tanjau Univ Hosp Graz, Dept Dermatol & Venereol, Graz, Austria.4 aut
7001 a Niederwieser, Dietgeru Univ Hosp Leipzig, Leipzig, Germany.4 aut
7001 a Hartmann, Karinu Univ Basel, Dept Dermatol, Div Allergy, Basel, Switzerland.;Uni Hosp Basel, Dept Biomed, Basel, Switzerland.;Univ Basel, Basel, Switzerland.4 aut
7001 a Triggiani, Massimou Univ Salerno, Div Allergy & Clin Immunol, Salerno, Italy.4 aut
7001 a Gotlib, Jasonu Stanford Univ, Sch Med, Dept Med, Div Hematol, Stanford, CA 94305 USA.4 aut
7001 a Hermine, Olivieru Univ Paris 05, Hop Necker, AP HP, Imagine Inst,French Reference Ctr Mastocytosis CE, Paris, France.4 aut
7001 a Arock, Michelu Hop La Pitie Salpetriere, Lab Hematol, Paris, France.4 aut
7001 a Kluin-Nelemans, Hanneke C.u Univ Groningen, Univ Med Ctr Groningen, Dept Hematol, Groningen, Netherlands.4 aut
7001 a Panse, Jensu Univ Hosp RWTH Aachen, Dept Oncol Haematol Haemostaseol & Stem Cell Tran, Aachen, Germany.;Aachen Bonn Cologne Dusseldorf CIO ABCD, Ctr Integrated Oncol, Aachen, Germany.4 aut
7001 a Sperr, Wolfgang R.u Med Univ Vienna, Dept Int Med 1, Div Hematol & Hemostaseol, Vienna, Austria.;Med Univ Vienna, Ludwig Boltzmann Inst Hematol & Oncol, Vienna, Austria.4 aut
7001 a Valent, Peter4 aut
7001 a Reiter, Andreasu Heidelberg Univ, Univ Hosp Mannheim, Dept Hematol & Oncol, Mannheim, Germany.4 aut
7001 a Jawhar, Mohamadu Heidelberg Univ, Univ Hosp Mannheim, Dept Hematol & Oncol, Mannheim, Germany.4 aut
7102 a Heidelberg Univ, Univ Hosp Mannheim, Dept Hematol & Oncol, Mannheim, Germany.b Univ Hosp RWTH Aachen, Dept Oncol Haematol Haemostaseol & Stem Cell Tran, Aachen, Germany.4 org
7730 t Journal of Allergy and Clinical Immunologyd : Elsevierg 11:2, s. 581-590q 11:2<581-590x 2213-2198x 2213-2201
8564 8u https://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-507040
8564 8u https://doi.org/10.1016/j.jaip.2022.10.051
8564 8u http://kipublications.ki.se/Default.aspx?queryparsed=id:152841587

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