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Clinical Impact of Skin Lesions in Mastocytosis: A Multicenter Study of the European Competence Network on Mastocytosis
E. Aberer, WR. Sperr, A. Bretterklieber, A. Avian, E. Hadzijusufovic, HC. Kluin-Nelemans, H. Oude Elberink, B. van Anrooij, M. Niedoszytko, M. Lange, A. Górska, C. Elena, V. Brazzelli, A. Belloni Fortina, F. Caroppo, K. Hartmann, A. Illerhaus, A....
Jazyk angličtina Země Spojené státy americké
Typ dokumentu časopisecké články, multicentrická studie, pozorovací studie, práce podpořená grantem
NLK
Free Medical Journals
od 1938 do Před 1 rokem
Freely Accessible Science Journals
od 1938 do Před 1 rokem
Open Access Digital Library
od 1938-01-01
- MeSH
- analýza přežití MeSH
- biopsie MeSH
- časové faktory MeSH
- diferenciální diagnóza MeSH
- dítě MeSH
- dospělí MeSH
- kojenec MeSH
- kostní dřeň patologie MeSH
- kožní mastocytóza diagnóza epidemiologie patologie MeSH
- kůže patologie MeSH
- lidé středního věku MeSH
- lidé MeSH
- mastocyty patologie MeSH
- mladiství MeSH
- mladý dospělý MeSH
- předškolní dítě MeSH
- prognóza MeSH
- registrace statistika a číselné údaje MeSH
- senioři MeSH
- systémová mastocytóza diagnóza mortalita patologie MeSH
- Check Tag
- dítě MeSH
- dospělí MeSH
- kojenec MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladiství MeSH
- mladý dospělý MeSH
- mužské pohlaví MeSH
- předškolní dítě MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- multicentrická studie MeSH
- pozorovací studie MeSH
- práce podpořená grantem MeSH
Mastocytosis is a rare neoplasm characterized by the expansion and accumulation of mast cells in various organ systems. Systemic mastocytosis (SM) may or may not present with cutaneous lesions. To examine the frequency and clinical impact of cutaneous involvement, data on 1,510 patients with mastocytosis collected in the registry of the European Competence Network on Mastocytosis were analyzed. Cutaneous involvement was found in 1,195 of 1,510 patients (79.1%). Of these, 286 had cutaneous mastocytosis, and 721 had SM with skin involvement. Adult patients with skin involvement who did not have a bone marrow examination (n = 188) were defined as having mastocytosis in the skin. In 315 patients, SM without skin involvement was found. The percentage of cases with cutaneous involvement was higher in indolent SM (100%) and smoldering SM (87.9%) compared to aggressive SM (46.8%) or mast cell leukemia (38.5%). After a median follow-up of 5.6 years, no patient with cutaneous mastocytosis had died, but 2.6% of the patients with mastocytosis in the skin, 5.7% of the patients with SM with skin involvement, and 28.95% of the patients with SM without skin involvement had died. Overall survival was longer in patients with skin involvement (cutaneous mastocytosis and/or mastocytosis in the skin and/or SM with skin involvement) than in patients with SM without skin involvement (P < 0.0001). These data argue for a thorough examination of both the skin and bone marrow in adult patients with mastocytosis.
Allergy Unit Verona University Hospital Verona Italy
CEITEC and University Hospital Brno Czech Republic
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 Hematology and Oncology University of Pavia Pavia Italy
Department of Internal Medicine 4 Oncology Hematology Hemostaseology University Halle Germany
Department of Molecular Medicine University of Pavia Pavia Italy
Division of Allergy Department of Dermatology University of Basel Basel Switzerland
Fondazione IRCCS Policlinico San Matteo Pavia Italy
Hematology Unit Verona University Hospital Verona Italy
Ludwig Boltzmann Institute for Hematology and Oncology Medical University of Vienna Vienna Austria
Pediatric Dermatology Unit Department of Medicine University of Padova Padova Italy
Citace poskytuje Crossref.org
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- $a Mastocytosis is a rare neoplasm characterized by the expansion and accumulation of mast cells in various organ systems. Systemic mastocytosis (SM) may or may not present with cutaneous lesions. To examine the frequency and clinical impact of cutaneous involvement, data on 1,510 patients with mastocytosis collected in the registry of the European Competence Network on Mastocytosis were analyzed. Cutaneous involvement was found in 1,195 of 1,510 patients (79.1%). Of these, 286 had cutaneous mastocytosis, and 721 had SM with skin involvement. Adult patients with skin involvement who did not have a bone marrow examination (n = 188) were defined as having mastocytosis in the skin. In 315 patients, SM without skin involvement was found. The percentage of cases with cutaneous involvement was higher in indolent SM (100%) and smoldering SM (87.9%) compared to aggressive SM (46.8%) or mast cell leukemia (38.5%). After a median follow-up of 5.6 years, no patient with cutaneous mastocytosis had died, but 2.6% of the patients with mastocytosis in the skin, 5.7% of the patients with SM with skin involvement, and 28.95% of the patients with SM without skin involvement had died. Overall survival was longer in patients with skin involvement (cutaneous mastocytosis and/or mastocytosis in the skin and/or SM with skin involvement) than in patients with SM without skin involvement (P < 0.0001). These data argue for a thorough examination of both the skin and bone marrow in adult patients with mastocytosis.
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