Clinical Features and Survival of Patients with Indolent Systemic Mastocytosis defined by the Updated WHO Classification,Allergy

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Clinical Features and Survival of Patients with Indolent Systemic Mastocytosis defined by the Updated WHO Classification
Allergy ( IF 12.4 ) Pub Date : 2020-03-16 , DOI: 10.1111/all.14248
Jakub Trizuljak ₁ , Wolfgang R Sperr ₂ , Lucie Nekvindová ₃ , Hanneke O Elberink ₄ , Karoline V Gleixner ₂ , Aleksandra Gorska ₅ , Magdalena Lange ₆ , Karin Hartmann _{7,

8} , Anja Illerhaus ₈ , Massimiliano Bonifacio ₉ , Cecelia Perkins ₁₀ , Chiara Elena ₁₁ , Luca Malcovati ₁₁ , Anna B Fortina ₁₂ , Khalid Shoumariyeh ₁₃ , Mohamad Jawhar ₁₄ , Roberta Zanotti ₉ , Patrizia Bonadonna ₁₅ , Francesca Caroppo ₁₂ , Alexander Zink ₁₆ , Massimo Triggiani ₁₇ , Roberta Parente ₁₇ , Nikolas von Bubnoff ₁₃ , Akif S Yavuz ₁₈ , Hans Hägglund ₁₉ , Mattias Mattsson ₁₉ , Jens Panse ₂₀ , Nadja Jäkel ₂₁ , Alex Kilbertus ₂₂ , Olivier Hermine ₂₃ , Michel Arock ₂₄ , David Fuchs ₂₅ , Vito Sabato ₂₆ , Knut Brockow ₁₆ , Agnes Bretterklieber ₂₇ , Marek Niedoszytko ₅ , Björn van Anrooij ₄ , Andreas Reiter ₁₄ , Jason Gotlib ₁₀ , Hanneke C Kluin-Nelemans ₂₈ , Jiri Mayer ₁ , Michael Doubek ₁ , Peter Valent ₂

Affiliation

Department of Hematology and Oncology, University Hospital, CEITEC Masaryk University, Brno, Czech Republic.
Department of Internal Medicine I, Division of Hematology and Hemostaseology, Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Vienna, Austria.
Institute of Biostatistics and Analyses, Ltd., Spinoff Company of Masaryk University, Brno, Czech Republic.
Department of Allergology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
Department of Allergology, Medical University of Gdańsk, Gdańsk, Poland.
Department of Dermatology, Venereology and Allergology, Medical University of Gdańsk, Gdańsk, Poland.
Division of Allergy, Department of Dermatology, University of Basel, Basel, Switzerland.
Department of Dermatology, University of Cologne, Cologne, Germany.
Department of Medicine, Section of Hematology, University of Verona, Verona, Italy.
Division of Hematology, Department of Medicine, Stanford University, School of Medicine/Stanford Cancer Institute, Stanford, CA, USA.
Division of Hematology, Fondazione IRCCS Policlinico San Matteo & University of Pavia, Pavia, Italy.
Pediatric Dermatology Unit, Department of Medicine, University of Padova, Padova, Italy.
Department of Hematology, Oncology and Stem Cell Transplantation, Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany.
Hämatologie und Onkologie, III. Medizinische Klinik, Universitätsmedizin Mannheim, Universität Heidelberg, Mannheim, Germany.
Allergy Unit, Verona University Hospital, Verona, Italy.
Department of Dermatology and Allergy Biederstein, School of Medicine, Technical University of Munich, Munich, Germany.
Division of Allergy and Clinical Immunology, University of Salerno, Salerno, Italy.
Division of Hematology, Istanbul Medical School, University of Istanbul, Istanbul, Turkey.
Department of Medical Sciences, Uppsala University, Section of Hematology, Uppsala University Hospital, Uppsala, Sweden.
Department of Oncology, Haematology, Haemostaseology and Stem Cell Transplantation, University Hospital RWTH Aachen, Aachen, Germany.
Department of Internal Medicine IV, University Hospital Halle, Halle, Germany.
Department of Dermatology and Venereology, Kepler University Hospital, Johannes Kepler University, Linz, Austria.
Departement of Hematology, Centre National de Référence des Mastocytoses, Imagine Institute, INSERM U1123, Université Paris Descartes, Sorbonne, Paris Cité, Hôpital Necker, Assistance Publique des Hôpitaux de Paris (APHP), Paris, France.
Laboratory of Hematology, Pitié-Salpêtrière Hospital, Paris, France.
Department of Internal Medicine 3, Hematology and Oncology, Kepler University Hospital, Johannes Kepler University, Linz, Austria.
Faculty of Medicine and Health Sciences, Department of Immunology-Allergology-Rheumatology, University of Antwerp and Antwerp University Hospital, Antwerp, Belgium.
Department of Dermatology and Venereology, Medical University of Graz, Graz, Austria.
Department of Hematology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.

In indolent systemic mastocytosis (ISM), several risk factors of disease progression have been identified. Previous studies, performed with limited patient numbers, have also shown that the clinical course in ISM is stable and comparable to that of cutaneous mastocytosis (CM). The aim of this project was to compare the prognosis of patients with ISM with that of patients with CM.

中文翻译：

更新后的 WHO 分类定义的惰性系统性肥大细胞增多症患者的临床特征和生存期

在惰性系统性肥大细胞增多症 (ISM) 中，已经确定了几种疾病进展的危险因素。先前在有限患者数量的情况下进行的研究也表明，ISM 的临床过程是稳定的，可与皮肤肥大细胞增多症 (CM) 的临床过程相媲美。该项目的目的是比较 ISM 患者与 CM 患者的预后。

更新日期：2020-03-16

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