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Prognostic Impact of Organomegaly in Mastocytosis: An Analysis of the European Competence Network on Mastocytosis
J. Lübke, J. Schwaab, D. Christen, HO. Elberink, B. Span, M. Niedoszytko, A. Gorska, M. Lange, KV. Gleixner, E. Hadzijusufovic, O. Solomianyi, I. Angelova-Fischer, R. Zanotti, M. Bonifacio, P. Bonadonna, K. Shoumariyeh, N. von Bubnoff, S. Müller,...
Language English Country United States
Document type Journal Article, Research Support, Non-U.S. Gov't
- MeSH
- Mastocytosis, Cutaneous * MeSH
- Humans MeSH
- Lymphadenopathy * MeSH
- Mastocytosis * diagnosis MeSH
- Prognosis MeSH
- Disease Progression MeSH
- Mastocytosis, Systemic * diagnosis epidemiology MeSH
- Check Tag
- Humans MeSH
- Publication type
- Journal Article MeSH
- Research Support, Non-U.S. Gov't MeSH
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.
Allergy Unit Verona University Hospital Verona Italy
Center for Integrated Oncology Aachen Bonn Cologne Düsseldorf Aachen Germany
Department of Allergology Medical University of Gdańsk Gdańsk Poland
Department of Biomedicine University Hospital Basel and University of Basel Basel Switzerland
Department of Dermatology and Venereology University Hospital Graz Graz Austria
Department of Dermatology Faculty of Medicine Medical Center University of Freiburg Freiburg Germany
Department of Dermatology Medical University of Gdańsk Gdańsk Poland
Department of Hematology Semmelweis University Budapest Hungary
Division of Allergy and Clinical Immunology University of Salerno Salerno Italy
Division of Allergy Department of Dermatology University of Basel Basel Switzerland
Division of Hematology Department of Medical Sciences Uppsala University Uppsala Sweden
Division of Hematology Istanbul Medical School University of Istanbul Istanbul Turkey
German Cancer Consortium Partner Site Freiburg Freiburg Germany
Laboratory of Hematology Pitié Salpêtrière Hospital Paris France
Ludwig Boltzmann Institute for Hematology and Oncology Medical University of Vienna Vienna Austria
Pediatric Dermatology Unit Department of Medicine University of Padova Padova Italy
Section of Hematology Department of Medicine Verona University Hospital Verona Italy
References provided by Crossref.org
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- $a Lübke, Johannes $u Department of Hematology and Oncology, University Hospital Mannheim, Heidelberg University, Mannheim, Germany
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- $a Prognostic Impact of Organomegaly in Mastocytosis: An Analysis of the European Competence Network on Mastocytosis / $c J. Lübke, J. Schwaab, D. Christen, HO. Elberink, B. Span, M. Niedoszytko, A. Gorska, M. Lange, KV. Gleixner, E. Hadzijusufovic, O. Solomianyi, I. Angelova-Fischer, R. Zanotti, M. Bonifacio, P. Bonadonna, K. Shoumariyeh, N. von Bubnoff, S. Müller, C. Perkins, C. Elena, L. Malcovati, H. Hagglund, M. Mattsson, R. Parente, J. Varkonyi, AB. Fortina, F. Caroppo, A. Zink, K. Brockow, C. Breynaert, D. Bullens, AS. Yavuz, M. Doubek, V. Sabato, T. Schug, D. Niederwieser, K. Hartmann, M. Triggiani, J. Gotlib, O. Hermine, M. Arock, HC. Kluin-Nelemans, J. Panse, WR. Sperr, P. Valent, A. Reiter, M. Jawhar
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- $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.
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