Scoring the Risk of Having Systemic Mastocytosis in Adult Patients with Mastocytosis in the Skin.

Adult Bone Marrow Female Humans Male Mast Cells Mastocytosis Mastocytosis, Cutaneous / diagnosis Mastocytosis, Systemic / diagnosis Middle Aged Tryptases
Cutaneous mastocytosis Mast cell disease Mastocytosis Risk score Systemic mastocytosis Tryptase

Journal

The journal of allergy and clinical immunology. In practice
ISSN: 2213-2201
Titre abrégé: J Allergy Clin Immunol Pract
Pays: United States
ID NLM: 101597220

Informations de publication

Date de publication:
04 2021
Historique:
received: 13 07 2020
revised: 05 12 2020
accepted: 07 12 2020
pubmed: 22 12 2020
medline: 25 5 2021
entrez: 21 12 2020
Statut: ppublish

Résumé

Mastocytosis in adults often presents with skin lesions. A bone marrow biopsy is necessary to confirm or exclude the presence of systemic mastocytosis (SM) in these cases. When a bone marrow biopsy is not performed, the provisional diagnosis is mastocytosis in the skin (MIS). No generally accepted scoring system has been established to estimate the risk of SM in these patients. To develop a risk score to predict SM in adults with MIS. We examined 1145 patients with MIS from the European Competence Network on Mastocytosis Registry who underwent a bone marrow biopsy. A total of 944 patients had SM and 201 patients had cutaneous mastocytosis; 63.7% were female, and 36.3% were male. Median age was 44 ± 13.3 years. The median serum tryptase level amounted to 29.3 ± 81.9 ng/mL. We established a multivariate regression model using the whole population of patients as a training and validation set (bootstrapping). A risk score was developed and validated with receiver-operating curves. In the multivariate model, the tryptase level (P < .001), constitutional/cardiovascular symptoms (P = .014), and bone symptoms/osteoporosis (P < .001) were independent predictors of SM (P < .001; sensitivity, 90.7%; specificity, 69.1%). A 6-point risk score was established (risk, 10.7%-98.0%) and validated. Using a large data set of the European Competence Network on Mastocytosis Registry, we created a risk score to predict the presence of SM in patients with MIS. Although the score will need further validation in independent cohorts, our score seems to discriminate safely between patients with SM and with pure cutaneous mastocytosis.

Sections du résumé

BACKGROUND
Mastocytosis in adults often presents with skin lesions. A bone marrow biopsy is necessary to confirm or exclude the presence of systemic mastocytosis (SM) in these cases. When a bone marrow biopsy is not performed, the provisional diagnosis is mastocytosis in the skin (MIS). No generally accepted scoring system has been established to estimate the risk of SM in these patients.
OBJECTIVE
To develop a risk score to predict SM in adults with MIS.
METHODS
We examined 1145 patients with MIS from the European Competence Network on Mastocytosis Registry who underwent a bone marrow biopsy. A total of 944 patients had SM and 201 patients had cutaneous mastocytosis; 63.7% were female, and 36.3% were male. Median age was 44 ± 13.3 years. The median serum tryptase level amounted to 29.3 ± 81.9 ng/mL. We established a multivariate regression model using the whole population of patients as a training and validation set (bootstrapping). A risk score was developed and validated with receiver-operating curves.
RESULTS
In the multivariate model, the tryptase level (P < .001), constitutional/cardiovascular symptoms (P = .014), and bone symptoms/osteoporosis (P < .001) were independent predictors of SM (P < .001; sensitivity, 90.7%; specificity, 69.1%). A 6-point risk score was established (risk, 10.7%-98.0%) and validated.
CONCLUSIONS
Using a large data set of the European Competence Network on Mastocytosis Registry, we created a risk score to predict the presence of SM in patients with MIS. Although the score will need further validation in independent cohorts, our score seems to discriminate safely between patients with SM and with pure cutaneous mastocytosis.

Identifiants

DOI: 10.1016/j.jaip.2020.12.022 PMID: 33346151
pubmed: 33346151
pii: S2213-2198(20)31354-4
doi: 10.1016/j.jaip.2020.12.022
pii:
doi:

Substances chimiques

Tryptases EC 3.4.21.59

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

1705-1712.e4

Informations de copyright

Copyright © 2020 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.

Auteurs

David Fuchs (D)

Department for Hematology and Internal Oncology, Kepler University Hospital, Linz, Austria; Johannes Kepler University, Linz, Austria. Electronic address: mail@davidfuchs.at.

Alex Kilbertus (A)

Department of Dermatology and Venerology, Kepler University Hospital, Johannes Kepler University, Linz, Austria.

Karin Kofler (K)

Department for Hematology and Internal Oncology, Kepler University Hospital, Linz, Austria.

Nikolas von Bubnoff (N)

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

Khalid Shoumariyeh (K)

Department of Medicine I, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany; German Cancer Consortium (DKTK) Partner site Freiburg, Freiburg, Germany.

Roberta Zanotti (R)

Section of Hematology, Department of Medicine, Verona University Hospital, Verona, Italy.

Patrizia Bonadonna (P)

Allergy Unit, Verona University Hospital, Verona, Italy.

Luigi Scaffidi (L)

Section of Hematology, Department of Medicine, Verona University Hospital, Verona, Italy.

Michael Doubek (M)

University Hospital Brno, Brno, Czech Republic.

Hanneke Oude Elberink (HO)

Department of Internal Medicine, Section of Allergy, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.

Lambert F R Span (LFR)

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

Olivier Hermine (O)

Imagine Institute Université Paris Descartes, Sorbonne, Paris Cité, Centre national de référence des mastocytoses, Paris, France.

Chiara Elena (C)

Department of Hematology Oncology, IRCCS Policlinico San Matteo Foundation Pavia, Pavia, Italy.

Pietro Benvenuti (P)

Department of Hematology Oncology, School of Hematology, University of Pavia and IRCCS Policlinico San Matteo Foundation, Pavia, Italy.

Akif Selim Yavuz (AS)

Division of Hematology, Department of Internal Medicine, University of Istanbul, Istanbul, Turkey.

Knut Brockow (K)

Department of Dermatology and Allergy Biederstein, School of Medicine, Technical University of Munich, Munich, Germany.

Alexander Zink (A)

Department of Dermatology and Allergy Biederstein, School of Medicine, Technical University of Munich, Munich, Germany.

Elisabeth Aberer (E)

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

Aleksandra Gorska (A)

Department of Allergology, Medical University of Gdańsk, Gdańsk, Poland.

Jan Romantowski (J)

Department of Allergology, Medical University of Gdańsk, Gdańsk, Poland.

Emir Hadzijusufovic (E)

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.

Anna Belloni Fortina (AB)

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

Francesca Caroppo (F)

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

Cecelia Perkins (C)

Stanford Cancer Institute/Stanford University School of Medicine, Stanford, Calif.

Anja Illerhaus (A)

Department of Dermatology, University of Cologne, Cologne, Germany.

Jens Panse (J)

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

Vladan Vucinic (V)

University Hospital of Leipzig, Leipzig, Germany.

Mohamad Jawhar (M)

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

Vito Sabato (V)

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

Massimo Triggiani (M)

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

Roberta Parente (R)

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

Anna Bergström (A)

Department of Dermatology and Venereology, Akademiska University Hospital, Uppsala, and Department of Medical Sciences, Uppsala University, Uppsala, Sweden.

Christine Breynaert (C)

Department of General Internal Medicine - Allergy and Clinical Immunology (MASTEL), University Hospitals Leuven and KU Leuven Department of Microbiology, Immunology and Transplantation, Allergy and Clinical Immunology Research Group, Leuven, Belgium.

Jason Gotlib (J)

Stanford Cancer Institute/Stanford University School of Medicine, Stanford, Calif.

Andreas Reiter (A)

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

Karin Hartmann (K)

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

Marek Niedoszytko (M)

Department of Allergology, Medical University of Gdańsk, Gdańsk, Poland.

Michel Arock (M)

Department of Hematological Biology, Pitié-Salpêtrière Hospital, Paris Sorbonne University, Paris, France.

Hanneke C Kluin-Nelemans (HC)

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

Wolfgang R Sperr (WR)

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.

Rosemarie Greul (R)

Department for Hematology and Internal Oncology, Kepler University Hospital, Linz, Austria.

Peter Valent (P)

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.

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Classifications MeSH

questionsmedicales.fr › Personnes › Tranches d'âge › Adulte › Scoring the Risk of Having Systemic...
questionsmedicales.fr › Systèmes sanguin et immunitaire › Système immunitaire › Moelle osseuse › Scoring the Risk of Having Systemic...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Primates › Haplorhini › Catarrhini › Hominidae › Humains › Scoring the Risk of Having Systemic...
questionsmedicales.fr › Systèmes sanguin et immunitaire › Système immunitaire › Mastocytes › Scoring the Risk of Having Systemic...
questionsmedicales.fr › Maladies du système immunitaire › Troubles de l'activation mastocytaire › Mastocytose › Scoring the Risk of Having Systemic...
questionsmedicales.fr › Maladies du système immunitaire › Troubles de l'activation mastocytaire › Mastocytose › Mastocytose cutanée › Scoring the Risk of Having Systemic...
questionsmedicales.fr › Maladies du système immunitaire › Troubles de l'activation mastocytaire › Mastocytose › Mastocytose généralisée › Scoring the Risk of Having Systemic...
questionsmedicales.fr › Personnes › Tranches d'âge › Adulte › Adulte d'âge moyen › Scoring the Risk of Having Systemic...
questionsmedicales.fr › Enzymes et coenzymes › Enzymes › Hydrolases › Peptide hydrolases › Protéases à sérine › Serine endopeptidases › Tryptases › Scoring the Risk of Having Systemic...