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

Elisabeth Aberer¹, Wolfgang R Sperr², Agnes Bretterklieber¹, Alexander Avian³, Emir Hadzijusufovic⁴, Hanneke C Kluin-Nelemans⁵, Hanneke Oude Elberink⁶, Björn van Anrooij⁷, Marek Niedoszytko⁸, Magdalena Lange⁹, Aleksandra Górska⁸, Chiara Elena¹⁰, Valeria Brazzelli¹¹, Anna Belloni Fortina¹², Francesca Caroppo¹², Karin Hartmann¹³, Anja Illerhaus¹⁴, Andreas Reiter¹⁵, Mohamad Jawhar¹⁵, Patrizia Bonadonna¹⁶, Roberta Zanotti¹⁷, Massimo Triggiani¹⁸, Roberta Parente¹⁸, Jason Gotlib¹⁹, Michael Doubek²⁰, Nikolas von Bubnoff²¹, David Fuchs²², Vito Sabato²³, Knut Brockow²⁴, Nadja Jäkel²⁵, Jens Panse²⁶, Peter Valent²⁷

Affiliations

¹ Department of Dermatology and Venereology, Medical University of Graz, Graz, Austria.
² Department of Internal Medicine I, Division of Hematology and Hemostaseology, Medical University of Vienna, Vienna, Austria; Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Vienna, Austria.
³ Institute for Medical Informatics, Statistics and Documentation, Medical University of Graz, Graz, Austria.
⁴ Department of Internal Medicine I, Division of Hematology and Hemostaseology, Medical University of Vienna, Vienna, Austria; Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Vienna, Austria; Department/Hospital for Companion Animals and Horses, University Clinic for Small Animals, Internal Medicine Small Animals, University of Veterinary Medicine, Vienna, Austria.
⁵ Department of Hematology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
⁶ Department of Allergology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
⁷ Department of Hematology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands; Department of Allergology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
⁸ Department of Allergology, Medical University of Gdańsk, Gdańsk, Poland.
⁹ Department of Dermatology, Venereology and Allergology, Medical University of Gdańsk, Gdańsk, Poland.
¹⁰ Department of Molecular Medicine, University of Pavia, Pavia, Italy; Department of Hematology and Oncology, University of Pavia, Pavia, Italy.
¹¹ Department of Molecular Medicine, University of Pavia, Pavia, Italy; Department of Hematology and Oncology, University of Pavia, Pavia, Italy; Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
¹² Pediatric Dermatology Unit, Department of Medicine, University of Padova, Padova, Italy.
¹³ Division of Allergy, Department of Dermatology, University of Basel, Basel, Switzerland; Department of Dermatology, University of Cologne, Cologne, Germany.
¹⁴ Department of Dermatology, University of Cologne, Cologne, Germany.
¹⁵ Department of Hematology and Oncology, University Hospital Mannheim, Heidelberg University, Mannheim, Germany.
¹⁶ Allergy Unit, Verona University Hospital, Verona, Italy.
¹⁷ Hematology Unit, Verona University Hospital, Verona, Italy.
¹⁸ Division of Allergy and Clinical Immunology, Department of Medicine, University of Salerno, Salerno, Italy.
¹⁹ Division of Hematology, Department of Medicine, Stanford University School of Medicine, Stanford, California, USA.
²⁰ CEITEC and University Hospital, Brno, Czech Republic.
²¹ Department of Hematology, Oncology and Stem-Cell Transplantation, Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany; Department of Hematology and Oncology, Medical Center, University of Schleswig-Holstein, Campus Lübeck, Lübeck, Germany.
²² Department of Internal Medicine 3 - Hematology and Oncology, University Hospital, Johannes Kepler University, Linz, Austria.
²³ Department of Immunology-Allergology-Rheumatology, Faculty of Medicine and Health Sciences, Antwerp University Hospital, University of Antwerp and University Hospital, Antwerp, Belgium.
²⁴ Department of Dermatology and Allergology Biederstein, School of Medicine, Technical University of Munich, Munich, Germany.
²⁵ Department of Internal Medicine IV, Oncology, Hematology, Hemostaseology, University Halle (Saale), Halle (Saale), Germany.
²⁶ Department of Hematology, Hemostaseology, Oncology and Stem Cell Transplantation, University Hospital RWTH Aachen, Aachen, Germany.
²⁷ Department of Internal Medicine I, Division of Hematology and Hemostaseology, Medical University of Vienna, Vienna, Austria; Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Vienna, Austria. Electronic address: peter.valent@meduniwien.ac.at.

PMID: 33581142
DOI: 10.1016/j.jid.2020.12.030

Observational Study

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

Elisabeth Aberer et al. J Invest Dermatol. 2021 Jul.

. 2021 Jul;141(7):1719-1727.

doi: 10.1016/j.jid.2020.12.030. Epub 2021 Feb 11.

Authors

Affiliations

¹ Department of Dermatology and Venereology, Medical University of Graz, Graz, Austria.
² Department of Internal Medicine I, Division of Hematology and Hemostaseology, Medical University of Vienna, Vienna, Austria; Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Vienna, Austria.
³ Institute for Medical Informatics, Statistics and Documentation, Medical University of Graz, Graz, Austria.
⁴ Department of Internal Medicine I, Division of Hematology and Hemostaseology, Medical University of Vienna, Vienna, Austria; Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Vienna, Austria; Department/Hospital for Companion Animals and Horses, University Clinic for Small Animals, Internal Medicine Small Animals, University of Veterinary Medicine, Vienna, Austria.
⁵ Department of Hematology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
⁶ Department of Allergology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
⁷ Department of Hematology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands; Department of Allergology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
⁸ Department of Allergology, Medical University of Gdańsk, Gdańsk, Poland.
⁹ Department of Dermatology, Venereology and Allergology, Medical University of Gdańsk, Gdańsk, Poland.
¹⁰ Department of Molecular Medicine, University of Pavia, Pavia, Italy; Department of Hematology and Oncology, University of Pavia, Pavia, Italy.
¹¹ Department of Molecular Medicine, University of Pavia, Pavia, Italy; Department of Hematology and Oncology, University of Pavia, Pavia, Italy; Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
¹² Pediatric Dermatology Unit, Department of Medicine, University of Padova, Padova, Italy.
¹³ Division of Allergy, Department of Dermatology, University of Basel, Basel, Switzerland; Department of Dermatology, University of Cologne, Cologne, Germany.
¹⁴ Department of Dermatology, University of Cologne, Cologne, Germany.
¹⁵ Department of Hematology and Oncology, University Hospital Mannheim, Heidelberg University, Mannheim, Germany.
¹⁶ Allergy Unit, Verona University Hospital, Verona, Italy.
¹⁷ Hematology Unit, Verona University Hospital, Verona, Italy.
¹⁸ Division of Allergy and Clinical Immunology, Department of Medicine, University of Salerno, Salerno, Italy.
¹⁹ Division of Hematology, Department of Medicine, Stanford University School of Medicine, Stanford, California, USA.
²⁰ CEITEC and University Hospital, Brno, Czech Republic.
²¹ Department of Hematology, Oncology and Stem-Cell Transplantation, Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany; Department of Hematology and Oncology, Medical Center, University of Schleswig-Holstein, Campus Lübeck, Lübeck, Germany.
²² Department of Internal Medicine 3 - Hematology and Oncology, University Hospital, Johannes Kepler University, Linz, Austria.
²³ Department of Immunology-Allergology-Rheumatology, Faculty of Medicine and Health Sciences, Antwerp University Hospital, University of Antwerp and University Hospital, Antwerp, Belgium.
²⁴ Department of Dermatology and Allergology Biederstein, School of Medicine, Technical University of Munich, Munich, Germany.
²⁵ Department of Internal Medicine IV, Oncology, Hematology, Hemostaseology, University Halle (Saale), Halle (Saale), Germany.
²⁶ Department of Hematology, Hemostaseology, Oncology and Stem Cell Transplantation, University Hospital RWTH Aachen, Aachen, Germany.
²⁷ Department of Internal Medicine I, Division of Hematology and Hemostaseology, Medical University of Vienna, Vienna, Austria; Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Vienna, Austria. Electronic address: peter.valent@meduniwien.ac.at.

PMID: 33581142
DOI: 10.1016/j.jid.2020.12.030

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.

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

Affiliations

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

Authors

Affiliations

Abstract

Publication types

MeSH terms

Grants and funding