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

Feb 11, 2021

Contributor: 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

Mastocytosis is a rare neoplasm characterized by expansion and accumulation of mast cells in various organ systems. Systemic mastocytosis (SM) may or may not presents with cutaneous lesions. To examine the frequency and clinical impact of cutaneous involvement, 1510 mastocytosis patients collected in the registry of the European Competence Network on Mastocytosis were analyzed. Cutaneous involvement was found in 1195/1510 (79.1%) patients. Of these, 286 had cutaneous mastocytosis (CM) and 721 had SM with skin involvement (SM+). Adult patients with skin involvement who did not have a bone marrow examination (n=188) were defined as mastocytosis in the skin (MIS). In 315 patients with SM, no skin involvement was found (SM-). 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 CM had died, but 2.6% of the patients with MIS, 5.7% with SM+ and 28.95% with SM- had died. Overall survival was longer in patients with skin involvement (CM/MIS/SM+) compared to SM- patients (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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About The Expert

Elisabeth Aberer

Department of Dermatology and Venereology, Medical University of Graz, Austria.

Wolfgang R Sperr

Department of Internal Medicine I, Division of Hematology and Hemostaseology Medical University of Vienna, Austria;; Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Austria.

Agnes Bretterklieber

Department of Dermatology and Venereology, Medical University of Graz, Austria.

Alexander Avian

Institute for Medical Informatics, Statistics and Documentation, Medical University of Graz, Austria.

Emir Hadzijusufovic

Department of Internal Medicine I, Division of Hematology and Hemostaseology Medical University of Vienna, Austria;; Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Austria;; Department/Hospital for Companion Animals and Horses, University Clinic for Small Animals, Internal Medicine Small Animals, University of Veterinary Medicine, Vienna, Austria.

Hanneke C Kluin-Nelemans

Department of Hematology, University Medical Center Groningen, University of Groningen, The Netherlands.

Hanneke Oude Elberink

Department of Allergology, University Medical Center Groningen, University of Groningen, The Netherlands.

Björn van Anrooij

Department of Hematology, University Medical Center Groningen, University of Groningen, The Netherlands; Department of Allergology, University Medical Center Groningen, University of Groningen, The Netherlands.

Marek Niedoszytko

Department of Allergology, Medical University of Gdańsk, Poland.

Magdalena Lange

Department of Dermatology, Venereology and Allergology, Medical University of Gdańsk, Poland.

Aleksandra Górska

Department of Allergology, Medical University of Gdańsk, Poland.

Chiara Elena

Department of Molecular Medicine and Department of Hematology and Oncology, University of Pavia, Italy.

Valeria Brazzelli

Department of Molecular Medicine and Department of Hematology and Oncology, University of Pavia, Italy;; Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.

Anna Belloni Fortina

Pediatric Dermatology Unit, Department of Medicine, University of Padova, Padova, Italy.

Francesca Caroppo

Pediatric Dermatology Unit, Department of Medicine, University of Padova, Padova, Italy.

Karin Hartmann

Division of Allergy, Department of Dermatology, University of Basel, Switzerland;; Department of Dermatology, University of Cologne, Germany.

Anja Illerhaus

Department of Dermatology, University of Cologne, Germany.

Andreas Reiter

Department of Hematology and Oncology, University Hospital Mannheim, Heidelberg University, Mannheim, Germany.

Mohamad Jawhar

Department of Hematology and Oncology, University Hospital Mannheim, Heidelberg University, Mannheim, Germany.

Patrizia Bonadonna

Allergy Unit, Verona University Hospital, Verona, Italy.

Roberta Zanotti

Hematology Unit, Verona University Hospital, Verona, Italy.

Massimo Triggiani

Division of Allergy and Clinical Immunology, Department of Medicine, University of Salerno, Italy.

Roberta Parente

Division of Allergy and Clinical Immunology, Department of Medicine, University of Salerno, Italy.

Jason Gotlib

Division of Hematology, Department of Medicine, Stanford University School of Medicine, Stanford, CA, USA.

Michael Doubek

University Hospital and CEITEC, Brno, Czech Republic.

Nikolas von Bubnoff

Department of Hematology, Oncology and Stem Cell Transplantation, Medical Center, Faculty of Medicine, University of Freiburg, Germany;; Department of Hematology and Oncology, Medical Center, University of Schleswig-Holstein, Campus Lübeck, Germany.

David Fuchs

Department of Internal Medicine 3, Hematology and Oncology, University Hospital, Johannes Kepler University, Linz, Austria.

Vito Sabato

Department of Immunology-Allergology-Rheumatology, Faculty of Medicine and Health Sciences, University of Antwerp and University Hospital, Antwerp, Belgium.

Knut Brockow

Department of Dermatology und Allergology Biederstein, School of Medicine, Technical University of Munich, Germany.

Nadja Jäkel

Department of Internal Medicine IV, Oncology, Hematology, Hemostaseology, University Halle (Saale), Germany.

Jens Panse

Department of Hematology, Hemostaseology, Oncology and Stem Cell Transplantation University Hospital RWTH Aachen, Germany.

Peter Valent

Department of Internal Medicine I, Division of Hematology and Hemostaseology Medical University of Vienna, Austria;; Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Austria;. Electronic address: peter.valent@meduniwien.ac.at.

References

PubMed