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Refined diagnostic criteria for bone marrow mastocytosis: a proposal of the European competence network on mastocytosis
R. Zanotti, M. Bonifacio, G. Lucchini, WR. Sperr, L. Scaffidi, B. van Anrooij, HN. Oude Elberink, J. Rossignol, O. Hermine, A. Gorska, M. Lange, E. Hadzijusufovic, C. Miething, S. Müller, C. Perkins, W. Shomali, C. Elena, A. Illerhaus, M. Jawhar,...
Language English Country Great Britain
Document type Journal Article, Research Support, Non-U.S. Gov't
NLK
ProQuest Central
from 2000-01-01 to 1 year ago
Open Access Digital Library
from 1997-01-01
Nursing & Allied Health Database (ProQuest)
from 2000-01-01 to 1 year ago
Health & Medicine (ProQuest)
from 2000-01-01 to 1 year ago
Public Health Database (ProQuest)
from 2000-01-01 to 1 year ago
- MeSH
- Adult MeSH
- Bone Marrow metabolism pathology MeSH
- Skin Diseases physiopathology MeSH
- Middle Aged MeSH
- Humans MeSH
- Mastocytosis diagnosis epidemiology metabolism MeSH
- Mast Cells metabolism pathology MeSH
- Survival Rate MeSH
- Follow-Up Studies MeSH
- Prognosis MeSH
- Aged, 80 and over MeSH
- Aged MeSH
- Mastocytosis, Systemic diagnosis epidemiology metabolism MeSH
- Tryptases metabolism MeSH
- Check Tag
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Aged, 80 and over MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Research Support, Non-U.S. Gov't MeSH
- Geographicals
- Europe MeSH
In the current classification of the World Health Organization (WHO), bone marrow mastocytosis (BMM) is a provisional variant of indolent systemic mastocytosis (ISM) defined by bone marrow involvement and absence of skin lesions. However, no additional diagnostic criteria for BMM have been proposed. Within the registry dataset of the European Competence Network on Mastocytosis, we compared characteristics and outcomes of 390 patients with BMM and 1175 patients with typical ISM. BMM patients were significantly older, predominantly male, had lower tryptase and lower burden of neoplastic mast cells, and displayed a higher frequency of allergic reactions, mainly triggered by Hymenoptera, than patients with typical ISM. The estimated 10-year progression-free survival of BMM and typical ISM was 95.9% and 92.6%, respectively. In BMM patients defined by WHO-based criteria, the presence of one B-Finding and tryptase level ≥125 ng/mL were identified as risk factors for progression in multivariate analyses. BMM patients without any of these risk factors were found to have better progression-free survival (p < 0.05) and better overall survival (p < 0.05) than other ISM patients. These data support the proposal to define BMM as a separate SM variant characterized by SM criteria, absence of skin lesions, absence of B-Findings, and tryptase levels <125 ng/mL.
Allergy Unit Verona University Hospital Verona Italy
Biostatistical Service ASST of Mantova Mantua Italy
Department of Allergology Medical University of Gdańsk Gdańsk Poland
Department of Dermatology and Allergy Biederstein Technical University of Munich Munich Germany
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 Hematology and Internal Medicine Semmelweis University Budapest Hungary
Department of Hematology and Oncology Medical Center University of Schleswig Holstein Lübeck Germany
Department of Medicine Section of Hematology University of Verona Verona Italy
Department of Molecular Medicine University of Pavia Pavia Italy
Division of Allergy and Clinical Immunology University of Salerno Salerno Italy
Division of Hematology Fondazione IRCCS San Matteo Pavia Italy
Division of Hematology Istanbul Medical School University of Istanbul Istanbul Turkey
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
References provided by Crossref.org
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- $a Zanotti, Roberta $u Department of Medicine, Section of Hematology, University of Verona, Verona, Italy
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- $a Refined diagnostic criteria for bone marrow mastocytosis: a proposal of the European competence network on mastocytosis / $c R. Zanotti, M. Bonifacio, G. Lucchini, WR. Sperr, L. Scaffidi, B. van Anrooij, HN. Oude Elberink, J. Rossignol, O. Hermine, A. Gorska, M. Lange, E. Hadzijusufovic, C. Miething, S. Müller, C. Perkins, W. Shomali, C. Elena, A. Illerhaus, M. Jawhar, R. Parente, F. Caroppo, O. Solomianyi, A. Zink, M. Mattsson, AS. Yavuz, J. Panse, J. Varkonyi, M. Doubek, V. Sabato, C. Breynaert, V. Vucinic, T. Schug, H. Hägglund, F. Wortmann, K. Brockow, I. Angelova-Fischer, A. Belloni Fortina, M. Triggiani, A. Reiter, K. Hartmann, L. Malcovati, J. Gotlib, K. Shoumariyeh, M. Niedoszytko, M. Arock, HC. Kluin-Nelemans, P. Bonadonna, P. Valent
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- $a In the current classification of the World Health Organization (WHO), bone marrow mastocytosis (BMM) is a provisional variant of indolent systemic mastocytosis (ISM) defined by bone marrow involvement and absence of skin lesions. However, no additional diagnostic criteria for BMM have been proposed. Within the registry dataset of the European Competence Network on Mastocytosis, we compar $a In the current classification of the World Health Organization WHO bone marrow mastocytosis BMM is a provisional variant of indolent systemic mastocytosis ISM defined by bone marrow involvement and absence of skin lesions However no additional diagnostic criteria for BMM have been proposed Within the registry dataset of the European Competence Network on Mastocytosis we compared characte $a In the current classification of the World Health Organization (WHO), bone marrow mastocytosis (BMM) is a provisional variant of indolent systemic mastocytosis (ISM) defined by bone marrow involvement and absence of skin lesions. However, no additional diagnostic criteria for BMM have been proposed. Within the registry dataset of the European Competence Network on Mastocytosis, we compared characteristics and outcomes of 390 patients with BMM and 1175 patients with typical ISM. BMM patients were significantly older, predominantly male, had lower tryptase and lower burden of neoplastic mast cells, and displayed a higher frequency of allergic reactions, mainly triggered by Hymenoptera, than patients with typical ISM. The estimated 10-year progression-free survival of BMM and typical ISM was 95.9% and 92.6%, respectively. In BMM patients defined by WHO-based criteria, the presence of one B-Finding and tryptase level ≥125 ng/mL were identified as risk factors for progression in multivariate analyses. BMM patients without any of these risk factors were found to have better progression-free survival (p < 0.05) and better overall survival (p < 0.05) than other ISM patients. These data support the proposal to define BMM as a separate SM variant characterized by SM criteria, absence of skin lesions, absence of B-Findings, and tryptase levels <125 ng/mL.
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