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Clinical features and survival of patients with indolent systemic mastocytosis defined by the updated WHO classification
J. Trizuljak, WR. Sperr, L. Nekvindová, HO. Elberink, KV. Gleixner, A. Gorska, M. Lange, K. Hartmann, A. Illerhaus, M. Bonifacio, C. Perkins, C. Elena, L. Malcovati, AB. Fortina, K. Shoumariyeh, M. Jawhar, R. Zanotti, P. Bonadonna, F. Caroppo, A....
Language English Country Denmark
Document type Journal Article, Research Support, Non-U.S. Gov't
PubMed
32108361
DOI
10.1111/all.14248
Knihovny.cz E-resources
- MeSH
- Bone Marrow MeSH
- Humans MeSH
- Mastocytosis * MeSH
- Mast Cells MeSH
- Prognosis MeSH
- World Health Organization MeSH
- Mastocytosis, Systemic * diagnosis epidemiology MeSH
- Check Tag
- Humans MeSH
- Male MeSH
- Publication type
- Journal Article MeSH
- Research Support, Non-U.S. Gov't MeSH
BACKGROUND: In indolent systemic mastocytosis (ISM), several risk factors of disease progression have been identified. Previous studies, performed with limited patient numbers, have also shown that the clinical course in ISM is stable and comparable to that of cutaneous mastocytosis (CM). The aim of this project was to compare the prognosis of patients with ISM with that of patients with CM. METHODS: We employed a dataset of 1993 patients from the registry of the European Competence Network on Mastocytosis (ECNM) to compare outcomes of ISM and CM. RESULTS: We found that overall survival (OS) is worse in ISM compared to CM. Moreover, in patients with typical ISM, bone marrow mastocytosis (BMM), and smoldering SM (SSM), 4.1% of disease progressions have been observed (4.9% of progressions in typical ISM group, 1.7% in BMM, and 9.4% in SSM). Progressions to advanced SM were observed in 2.9% of these patients. In contrast, six patients with CM (1.7%) converted to ISM and no definitive progression to advanced SM was found. No significant differences in OS and event-free survival (EFS) were found when comparing ISM, BMM, and SSM. Higher risk of both progression and death was significantly associated with male gender, worse performance status, and organomegaly. CONCLUSION: Our data confirm the clinical impact of the WHO classification that separates ISM from CM and from other SM variants.
Allergy Unit Verona University Hospital Verona Italy
Department of Allergology Medical University of Gdańsk Gdańsk Poland
Department of Dermatology and Venereology Medical University of Graz Graz Austria
Department of Dermatology University of Cologne Cologne Germany
Department of Dermatology Venereology and Allergology Medical University of Gdańsk Gdańsk Poland
Department of Internal Medicine 4 University Hospital Halle Halle Germany
Department of Medicine Section of Hematology University of Verona Verona Italy
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 Fondazione IRCCS Policlinico San Matteo and University of Pavia Pavia Italy
Division of Hematology Istanbul Medical School University of Istanbul Istanbul Turkey
Laboratory of Hematology Pitié Salpêtrière Hospital Paris France
Pediatric Dermatology Unit Department of Medicine University of Padova Padova Italy
References provided by Crossref.org
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- $a Clinical features and survival of patients with indolent systemic mastocytosis defined by the updated WHO classification / $c J. Trizuljak, WR. Sperr, L. Nekvindová, HO. Elberink, KV. Gleixner, A. Gorska, M. Lange, K. Hartmann, A. Illerhaus, M. Bonifacio, C. Perkins, C. Elena, L. Malcovati, AB. Fortina, K. Shoumariyeh, M. Jawhar, R. Zanotti, P. Bonadonna, F. Caroppo, A. Zink, M. Triggiani, R. Parente, N. von Bubnoff, AS. Yavuz, H. Hägglund, M. Mattsson, J. Panse, N. Jäkel, A. Kilbertus, O. Hermine, M. Arock, D. Fuchs, V. Sabato, K. Brockow, A. Bretterklieber, M. Niedoszytko, B. van Anrooij, A. Reiter, J. Gotlib, HC. Kluin-Nelemans, J. Mayer, M. Doubek, P. Valent
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- $a BACKGROUND: In indolent systemic mastocytosis (ISM), several risk factors of disease progression have been identified. Previous studies, performed with limited patient numbers, have also shown that the clinical course in ISM is stable and comparable to that of cutaneous mastocytosis (CM). The aim of this project was to compare the prognosis of patients with ISM with that of patients with CM. METHODS: We employed a dataset of 1993 patients from the registry of the European Competence Network on Mastocytosis (ECNM) to compare outcomes of ISM and CM. RESULTS: We found that overall survival (OS) is worse in ISM compared to CM. Moreover, in patients with typical ISM, bone marrow mastocytosis (BMM), and smoldering SM (SSM), 4.1% of disease progressions have been observed (4.9% of progressions in typical ISM group, 1.7% in BMM, and 9.4% in SSM). Progressions to advanced SM were observed in 2.9% of these patients. In contrast, six patients with CM (1.7%) converted to ISM and no definitive progression to advanced SM was found. No significant differences in OS and event-free survival (EFS) were found when comparing ISM, BMM, and SSM. Higher risk of both progression and death was significantly associated with male gender, worse performance status, and organomegaly. CONCLUSION: Our data confirm the clinical impact of the WHO classification that separates ISM from CM and from other SM variants.
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