Aberer, Elisabeth ORCID: 0000-0002-5533-9373, Sperr, Wolfgang R., Bretterklieber, Agnes, Avian, Alexander ORCID: 0000-0003-1084-5763, Hadzijusufovic, Emir ORCID: 0000-0001-7409-4204, Kluin-Nelemans, Hanneke C., Elberink, Hanneke Oude, van Anrooij, Bjoern, Niedoszytko, Marek ORCID: 0000-0003-1089-1911, Lange, Magdalena ORCID: 0000-0002-6967-1393, Gorska, Aleksandra, Elena, Chiara, Brazzelli, Valeria, Fortina, Anna Belloni ORCID: 0000-0001-5791-0775, Caroppo, Francesca, Hartmann, Karin ORCID: 0000-0002-4595-8226, Illerhaus, Anja, Reiter, Andreas, Jawhar, Mohamad, Bonadonna, Patrizia, Zanotti, Roberta, Triggiani, Massimo, Parente, Roberta, Gotlib, Jason, Doubek, Michael, von Bubnoff, Nikolas, Fuchs, David, Sabato, Vito, Brockow, Knut, Jaekel, Nadja, Panse, Jens and Valent, Peter ORCID: 0000-0003-0456-5095 (2021). Clinical Impact of Skin Lesions in Mastocytosis: A Multicenter Study of the European Competence Network on Mastocytosis. J. Invest. Dermatol., 141 (7). S. 1719 - 1728. NEW YORK: ELSEVIER SCIENCE INC. ISSN 1523-1747

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Abstract

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 masto-cytosis 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.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Aberer, ElisabethUNSPECIFIEDorcid.org/0000-0002-5533-9373UNSPECIFIED
Sperr, Wolfgang R.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bretterklieber, AgnesUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Avian, AlexanderUNSPECIFIEDorcid.org/0000-0003-1084-5763UNSPECIFIED
Hadzijusufovic, EmirUNSPECIFIEDorcid.org/0000-0001-7409-4204UNSPECIFIED
Kluin-Nelemans, Hanneke C.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Elberink, Hanneke OudeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
van Anrooij, BjoernUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Niedoszytko, MarekUNSPECIFIEDorcid.org/0000-0003-1089-1911UNSPECIFIED
Lange, MagdalenaUNSPECIFIEDorcid.org/0000-0002-6967-1393UNSPECIFIED
Gorska, AleksandraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Elena, ChiaraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Brazzelli, ValeriaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Fortina, Anna BelloniUNSPECIFIEDorcid.org/0000-0001-5791-0775UNSPECIFIED
Caroppo, FrancescaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hartmann, KarinUNSPECIFIEDorcid.org/0000-0002-4595-8226UNSPECIFIED
Illerhaus, AnjaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Reiter, AndreasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Jawhar, MohamadUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bonadonna, PatriziaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Zanotti, RobertaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Triggiani, MassimoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Parente, RobertaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gotlib, JasonUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Doubek, MichaelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
von Bubnoff, NikolasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Fuchs, DavidUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Sabato, VitoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Brockow, KnutUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Jaekel, NadjaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Panse, JensUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Valent, PeterUNSPECIFIEDorcid.org/0000-0003-0456-5095UNSPECIFIED
URN: urn:nbn:de:hbz:38-606300
DOI: 10.1016/j.jid.2020.12.030
Journal or Publication Title: J. Invest. Dermatol.
Volume: 141
Number: 7
Page Range: S. 1719 - 1728
Date: 2021
Publisher: ELSEVIER SCIENCE INC
Place of Publication: NEW YORK
ISSN: 1523-1747
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
SYSTEMIC MASTOCYTOSIS; MAST-CELLS; PEDIATRIC MASTOCYTOSIS; URTICARIA PIGMENTOSA; CLASSIFICATION; DIAGNOSIS; DISEASE; ADULTS; RISKMultiple languages
DermatologyMultiple languages
URI: http://kups.ub.uni-koeln.de/id/eprint/60630

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