Clinical Impact of Skin Lesions in Mastocytosis: A Multicenter Study of the European Competence Network on Mastocytosis

Elisabeth Aberer; Wolfgang R. Sperr; Agnes Bretterklieber; Alexander Avian; Emir Hadzijusufovic; Hanneke C. Kluin-Nelemans; Hanneke Oude Elberink; Björn van Anrooij; Marek Niedoszytko; Magdalena Lange; Aleksandra Górska; Chiara Elena; Valeria Brazzelli; Anna Belloni Fortina; Francesca Caroppo; Karin Hartmann; Anja Illerhaus; Andreas Reiter; Mohamad Jawhar; Patrizia Bonadonna; Roberta Zanotti; Massimo Triggiani; Roberta Parente; Jason Gotlib; Michael Doubek; Nikolas von Bubnoff; David Fuchs; Vito Sabato; Knut Brockow; Nadja Jäkel; Jens Panse; Peter Valent

doi:10.1016/j.jid.2020.12.030

Clinical Impact of Skin Lesions in Mastocytosis: A Multicenter Study of the European Competence Network on Mastocytosis

Elisabeth Aberer, Wolfgang R. Sperr, Agnes Bretterklieber, Alexander Avian, Emir Hadzijusufovic, Hanneke C. Kluin-Nelemans, Hanneke Oude Elberink, Björn van Anrooij, Marek Niedoszytko, Magdalena Lange, Aleksandra Górska, Chiara Elena, Valeria Brazzelli, Anna Belloni Fortina, Francesca Caroppo, Karin Hartmann, Anja Illerhaus, Andreas Reiter, Mohamad Jawhar, Patrizia BonadonnaRoberta Zanotti, Massimo Triggiani, Roberta Parente, Jason Gotlib, Michael Doubek, Nikolas von Bubnoff, David Fuchs, Vito Sabato, Knut Brockow, Nadja Jäkel, Jens Panse, Peter Valent^*

^*Korrespondierende/r Autor/-in für diese Arbeit

26 Zitate (Scopus)

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 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.

Originalsprache	Englisch
Zeitschrift	Journal of Investigative Dermatology
Jahrgang	141
Ausgabenummer	7
Seiten (von - bis)	1719-1727
Seitenumfang	9
ISSN	0022-202X
DOIs	https://doi.org/10.1016/j.jid.2020.12.030
Publikationsstatus	Veröffentlicht - 07.2021

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This study was supported by the Austrian Science Fund (FWF) grants F4704-B20 and P32470-B. KH received a research grant from Euroimmun and support from intramural funding of the University of Basel , Basel, Switzerland. AI was supported by the Deutsche Forschungs-gemeinschaft (RA 2838) and by the Koeln Fortune Program, Faculty of Medicine, University of Cologne (Germany) (216/2016). VS is a senior clinical researcher of the Research Foundation Flanders/Fonds Wetenschappelijk Onderzoek (804518 N). We would like to thank all technicians, study coordinators, study nurses, and colleagues for data entry into the registry system. Our special thanks to data management and data controlling go to Nicole Cabral do O, Deborah Christen, Anne Simonowski, Stephanie Pulfer, Susanne Herndlhofer, and Karolin e V. Gleixner. We thank Dubravka Smiljkovic for her excellent technical assistance and her help in paper submission. This study was supported by the Austrian Science Fund (FWF) grants F4704-B20 and P32470-B. KH received a research grant from Euroimmun and support from intramural funding of the University of Basel, Basel, Switzerland. AI was supported by the Deutsche Forschungs-gemeinschaft (RA 2838) and by the Koeln Fortune Program, Faculty of Medicine, University of Cologne (Germany) (216/2016). VS is a senior clinical researcher of the Research Foundation Flanders/Fonds Wetenschappelijk Onderzoek (804518 N). We would like to thank all technicians, study coordinators, study nurses, and colleagues for data entry into the registry system. Our special thanks to data management and data controlling go to Nicole Cabral do O, Deborah Christen, Anne Simonowski, Stephanie Pulfer, Susanne Herndlhofer, and Karolin e V. Gleixner. We thank Dubravka Smiljkovic for her excellent technical assistance and her help in paper submission. Conceptualization: EA, WRS, PV; Data Curation: EA, WRS, AB, AA, EH, HCKN, HOE, BVA, MN, ML, AG, CE, VB, ABF, FC, KH, AI, AR, MJ, PB, RZ, MT, RP, JG, MD, NVB, DF, VS, KB, NJ, JP, PV; Formal Analysis: AA, WRS; Funding Acquisition: PV; Methodology: WRS, PV; Project Administration: PV; Supervision: EA, WRS, PV; Validation: EA, WRS, AB, AA, EH, HCKN, HOE, BVA, MN, ML, AG, CE, VB, ABF, FC, KH, AI, AR, MJ, PB, RZ, MT, RP, JG, MD, NVB, DF, VS, KB, NJ, JP, PV; Writing - Draft Preparation: EA, AB, PV; Writing - Review and Editing: EA, PV WRS received honoraria from Novartis, Pfizer, AbbVie, Daiichi Sankyo, Amgen, Thermo Fisher Scientific, Deciphera, Incyte, Celgene, and Jazz. HCKN received institutional financial support from Novartis to perform a phase II trial with midostaurin. BVA received financial support from Novartis for research and is on the advisory boards. ML recieved honoraria from Novartis. CE is on the advisory board of Novartis and Pfizer. VB is on the advisory boards with honoraria from Novartis and congresses with Janssen-Cilag Spa and Sanofi Genzyme. KH received honoraria (advisory board, consultant, and speaker) from ALK-Abelló , Blueprint Medicines, Deciphera, and Novartis. RZ is on the advisory board and received honoraria from Deciphera, Novartis, and Takeda. MT is on the advisory board and received honoraria from Deciphera and Novartis. JG is on the advisory board and received honoraria from Blueprint Medicines, Deciphera, and Allakos and received funding support to conduct clinical trials from Blueprint Medicines and Deciphera. MD has no conflict of interest within this study but received honoraria (advisory board, consultant, and speaker) from Novartis, AbbVie, Janssen-Cilag, Gilead Sciences, and AOP Orphan Pharmaceuticals outside of this study and received research grants from AbbVie, Janssen-Cilag, and Gilead. NVB received honoraria from Novartis and Bristol-Myers Squibb and research funding from Novartis. DF received honoraria from Novartis, Roche, and Pfizer and received a travel grant from Roche. NJ received honoraria from Novartis. JP is on the advisory board and received honoraria from Blueprint Medicines and Novartis; received honoraria from Alexion, Bristol-Myers Squibb, Boehringer Ingelheim, Grünenthal, Merck Sharp & Dohme, Novartis, Pfizer, and Chugai Pharmaceutical; and received funding support to conduct clinical trials from Blueprint Medicines and Deciphera. PV received honoraria (advisory board, consultant, and speaker) from Novartis, Deciphera, Blueprint Medicines, Celgene, and Pfizer and received research grants from Novarts, Celgene, and Pfizer. The remaining authors declared no conflict of interest.

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Aberer, E., Sperr, W. R., Bretterklieber, A., Avian, A., Hadzijusufovic, E., Kluin-Nelemans, H. C., Oude Elberink, H., van Anrooij, B., Niedoszytko, M., Lange, M., Górska, A., Elena, C., Brazzelli, V., Belloni Fortina, A., Caroppo, F., Hartmann, K., Illerhaus, A., Reiter, A., Jawhar, M., ... Valent, P. (2021). Clinical Impact of Skin Lesions in Mastocytosis: A Multicenter Study of the European Competence Network on Mastocytosis. Journal of Investigative Dermatology, 141(7), 1719-1727. https://doi.org/10.1016/j.jid.2020.12.030

@article{a0fd7035a0334f968ef1df3821c0c8f4,

title = "Clinical Impact of Skin Lesions in Mastocytosis: A Multicenter Study of the European Competence Network on Mastocytosis",

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 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.",

author = "Elisabeth Aberer and Sperr, \{Wolfgang R.\} and Agnes Bretterklieber and Alexander Avian and Emir Hadzijusufovic and Kluin-Nelemans, \{Hanneke C.\} and \{Oude Elberink\}, Hanneke and \{van Anrooij\}, Bj{\"o}rn and Marek Niedoszytko and Magdalena Lange and Aleksandra G{\'o}rska and Chiara Elena and Valeria Brazzelli and \{Belloni Fortina\}, Anna and Francesca Caroppo and Karin Hartmann and Anja Illerhaus and Andreas Reiter and Mohamad Jawhar and Patrizia Bonadonna and Roberta Zanotti and Massimo Triggiani and Roberta Parente and Jason Gotlib and Michael Doubek and \{von Bubnoff\}, Nikolas and David Fuchs and Vito Sabato and Knut Brockow and Nadja J{\"a}kel and Jens Panse and Peter Valent",

note = "Publisher Copyright: {\textcopyright} 2021 The Authors",

year = "2021",

month = jul,

doi = "10.1016/j.jid.2020.12.030",

language = "English",

volume = "141",

pages = "1719--1727",

journal = "Journal of Investigative Dermatology",

issn = "0022-202X",

publisher = "Elsevier B.V.",

number = "7",

}

Aberer, E, Sperr, WR, Bretterklieber, A, Avian, A, Hadzijusufovic, E, Kluin-Nelemans, HC, Oude Elberink, H, van Anrooij, B, Niedoszytko, M, Lange, M, Górska, A, Elena, C, Brazzelli, V, Belloni Fortina, A, Caroppo, F, Hartmann, K, Illerhaus, A, Reiter, A, Jawhar, M, Bonadonna, P, Zanotti, R, Triggiani, M, Parente, R, Gotlib, J, Doubek, M, von Bubnoff, N, Fuchs, D, Sabato, V, Brockow, K, Jäkel, N, Panse, J & Valent, P 2021, 'Clinical Impact of Skin Lesions in Mastocytosis: A Multicenter Study of the European Competence Network on Mastocytosis', Journal of Investigative Dermatology, Jg. 141, Nr. 7, S. 1719-1727. https://doi.org/10.1016/j.jid.2020.12.030

Clinical Impact of Skin Lesions in Mastocytosis: A Multicenter Study of the European Competence Network on Mastocytosis. / Aberer, Elisabeth; Sperr, Wolfgang R.; Bretterklieber, Agnes et al.
in: Journal of Investigative Dermatology, Jahrgang 141, Nr. 7, 07.2021, S. 1719-1727.

Publikation: Beiträge in Fachzeitschriften › Zeitschriftenaufsätze › Forschung › Begutachtung

TY - JOUR

T1 - Clinical Impact of Skin Lesions in Mastocytosis: A Multicenter Study of the European Competence Network on Mastocytosis

AU - Aberer, Elisabeth

AU - Sperr, Wolfgang R.

AU - Bretterklieber, Agnes

AU - Avian, Alexander

AU - Hadzijusufovic, Emir

AU - Kluin-Nelemans, Hanneke C.

AU - Oude Elberink, Hanneke

AU - van Anrooij, Björn

AU - Niedoszytko, Marek

AU - Lange, Magdalena

AU - Górska, Aleksandra

AU - Elena, Chiara

AU - Brazzelli, Valeria

AU - Belloni Fortina, Anna

AU - Caroppo, Francesca

AU - Hartmann, Karin

AU - Illerhaus, Anja

AU - Reiter, Andreas

AU - Jawhar, Mohamad

AU - Bonadonna, Patrizia

AU - Zanotti, Roberta

AU - Triggiani, Massimo

AU - Parente, Roberta

AU - Gotlib, Jason

AU - Doubek, Michael

AU - von Bubnoff, Nikolas

AU - Fuchs, David

AU - Sabato, Vito

AU - Brockow, Knut

AU - Jäkel, Nadja

AU - Panse, Jens

AU - Valent, Peter

PY - 2021/7

Y1 - 2021/7

N2 - 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.

AB - 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.

UR - https://www.scopus.com/pages/publications/85104126338

UR - https://www.mendeley.com/catalogue/9081a560-f3aa-3533-9b72-02c568ba4bf5/

U2 - 10.1016/j.jid.2020.12.030

DO - 10.1016/j.jid.2020.12.030

M3 - Journal articles

C2 - 33581142

AN - SCOPUS:85104126338

SN - 0022-202X

VL - 141

SP - 1719

EP - 1727

JO - Journal of Investigative Dermatology

JF - Journal of Investigative Dermatology

IS - 7

ER -

Clinical Impact of Skin Lesions in Mastocytosis: A Multicenter Study of the European Competence Network on Mastocytosis

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