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Clinical features and survival of patients with indolent systemic mastocytosis defined by the updated WHO classification.
Trizuljak, Jakub; Sperr, Wolfgang R; Nekvindová, Lucie; Elberink, Hanneke O; Gleixner, Karoline V; Gorska, Aleksandra; Lange, Magdalena; Hartmann, Karin; Illerhaus, Anja; Bonifacio, Massimiliano; Perkins, Cecelia; Elena, Chiara; Malcovati, Luca; Fortina, Anna B; Shoumariyeh, Khalid; Jawhar, Mohamad; Zanotti, Roberta; Bonadonna, Patrizia; Caroppo, Francesca; Zink, Alexander; Triggiani, Massimo; Parente, Roberta; von Bubnoff, Nikolas; Yavuz, Akif S; Hägglund, Hans; Mattsson, Mattias; Panse, Jens; Jäkel, Nadja; Kilbertus, Alex; Hermine, Olivier; Arock, Michel; Fuchs, David; Sabato, Vito; Brockow, Knut; Bretterklieber, Agnes; Niedoszytko, Marek; van Anrooij, Björn; Reiter, Andreas; Gotlib, Jason; Kluin-Nelemans, Hanneke C; Mayer, Jiri; Doubek, Michael; Valent, Peter.
Afiliação
  • Trizuljak J; Department of Hematology and Oncology, University Hospital, CEITEC Masaryk University, Brno, Czech Republic.
  • Sperr WR; Department of Internal Medicine I, Division of Hematology and Hemostaseology, Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Vienna, Austria.
  • Nekvindová L; Institute of Biostatistics and Analyses, Ltd., Spinoff Company of Masaryk University, Brno, Czech Republic.
  • Elberink HO; Department of Allergology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
  • Gleixner KV; Department of Internal Medicine I, Division of Hematology and Hemostaseology, Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Vienna, Austria.
  • Gorska A; Department of Allergology, Medical University of Gdansk, Gdansk, Poland.
  • Lange M; Department of Dermatology, Venereology and Allergology, Medical University of Gdansk, Gdansk, Poland.
  • Hartmann K; Division of Allergy, Department of Dermatology, University of Basel, Basel, Switzerland.
  • Illerhaus A; Department of Dermatology, University of Cologne, Cologne, Germany.
  • Bonifacio M; Department of Dermatology, University of Cologne, Cologne, Germany.
  • Perkins C; Department of Medicine, Section of Hematology, University of Verona, Verona, Italy.
  • Elena C; Division of Hematology, Department of Medicine, Stanford University, School of Medicine/Stanford Cancer Institute, Stanford, CA, USA.
  • Malcovati L; Division of Hematology, Fondazione IRCCS Policlinico San Matteo & University of Pavia, Pavia, Italy.
  • Fortina AB; Division of Hematology, Fondazione IRCCS Policlinico San Matteo & University of Pavia, Pavia, Italy.
  • Shoumariyeh K; Pediatric Dermatology Unit, Department of Medicine, University of Padova, Padova, Italy.
  • Jawhar M; Department of Hematology, Oncology and Stem Cell Transplantation, Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany.
  • Zanotti R; Hämatologie und Onkologie, III. Medizinische Klinik, Universitätsmedizin Mannheim, Universität Heidelberg, Mannheim, Germany.
  • Bonadonna P; Department of Medicine, Section of Hematology, University of Verona, Verona, Italy.
  • Caroppo F; Allergy Unit, Verona University Hospital, Verona, Italy.
  • Zink A; Pediatric Dermatology Unit, Department of Medicine, University of Padova, Padova, Italy.
  • Triggiani M; Department of Dermatology and Allergy Biederstein, School of Medicine, Technical University of Munich, Munich, Germany.
  • Parente R; Division of Allergy and Clinical Immunology, University of Salerno, Salerno, Italy.
  • von Bubnoff N; Division of Allergy and Clinical Immunology, University of Salerno, Salerno, Italy.
  • Yavuz AS; Department of Hematology, Oncology and Stem Cell Transplantation, Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany.
  • Hägglund H; Division of Hematology, Istanbul Medical School, University of Istanbul, Istanbul, Turkey.
  • Mattsson M; Department of Medical Sciences, Uppsala University, Section of Hematology, Uppsala University Hospital, Uppsala, Sweden.
  • Panse J; Department of Medical Sciences, Uppsala University, Section of Hematology, Uppsala University Hospital, Uppsala, Sweden.
  • Jäkel N; Department of Oncology, Haematology, Haemostaseology and Stem Cell Transplantation, University Hospital RWTH Aachen, Aachen, Germany.
  • Kilbertus A; Department of Internal Medicine IV, University Hospital Halle, Halle, Germany.
  • Hermine O; Department of Dermatology and Venereology, Kepler University Hospital, Johannes Kepler University, Linz, Austria.
  • Arock M; 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.
  • Fuchs D; Laboratory of Hematology, Pitié-Salpêtrière Hospital, Paris, France.
  • Sabato V; Department of Internal Medicine 3, Hematology and Oncology, Kepler University Hospital, Johannes Kepler University, Linz, Austria.
  • Brockow K; Faculty of Medicine and Health Sciences, Department of Immunology-Allergology-Rheumatology, University of Antwerp and Antwerp University Hospital, Antwerp, Belgium.
  • Bretterklieber A; Department of Dermatology and Allergy Biederstein, School of Medicine, Technical University of Munich, Munich, Germany.
  • Niedoszytko M; Department of Dermatology and Venereology, Medical University of Graz, Graz, Austria.
  • van Anrooij B; Department of Allergology, Medical University of Gdansk, Gdansk, Poland.
  • Reiter A; Department of Allergology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
  • Gotlib J; Hämatologie und Onkologie, III. Medizinische Klinik, Universitätsmedizin Mannheim, Universität Heidelberg, Mannheim, Germany.
  • Kluin-Nelemans HC; Division of Hematology, Department of Medicine, Stanford University, School of Medicine/Stanford Cancer Institute, Stanford, CA, USA.
  • Mayer J; Department of Hematology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
  • Doubek M; Department of Hematology and Oncology, University Hospital, CEITEC Masaryk University, Brno, Czech Republic.
  • Valent P; Department of Hematology and Oncology, University Hospital, CEITEC Masaryk University, Brno, Czech Republic.
Allergy ; 75(8): 1927-1938, 2020 08.
Article em En | MEDLINE | ID: mdl-32108361
ABSTRACT

BACKGROUND:

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.

METHODS:

We employed a dataset of 1993 patients from the registry of the European Competence Network on Mastocytosis (ECNM) to compare outcomes of ISM and CM.

RESULTS:

We found that overall survival (OS) is worse in ISM compared to CM. Moreover, in patients with typical ISM, bone marrow mastocytosis (BMM), and smoldering SM (SSM), 4.1% of disease progressions have been observed (4.9% of progressions in typical ISM group, 1.7% in BMM, and 9.4% in SSM). Progressions to advanced SM were observed in 2.9% of these patients. In contrast, six patients with CM (1.7%) converted to ISM and no definitive progression to advanced SM was found. No significant differences in OS and event-free survival (EFS) were found when comparing ISM, BMM, and SSM. Higher risk of both progression and death was significantly associated with male gender, worse performance status, and organomegaly.

CONCLUSION:

Our data confirm the clinical impact of the WHO classification that separates ISM from CM and from other SM variants.
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Texto completo: 1 Base de dados: MEDLINE Assunto principal: Mastocitose / Mastocitose Sistêmica Tipo de estudo: Diagnostic_studies / Prognostic_studies / Risk_factors_studies Limite: Humans / Male Idioma: En Ano de publicação: 2020 Tipo de documento: Article
Texto completo
Imprimir
XML
PubMed Links
Buscar no Google

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Mastocitose / Mastocitose Sistêmica Tipo de estudo: Diagnostic_studies / Prognostic_studies / Risk_factors_studies Limite: Humans / Male Idioma: En Ano de publicação: 2020 Tipo de documento: Article