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Mastocytosis pp 287-299 | Cite as

History and Current Status of Mastocytosis Research in the European Competence Network on Mastocytosis

  • Peter ValentEmail author
  • Michel Arock
  • Emir Hadzijusufovic
  • Wolfgang R. Sperr
  • Karin Hartmann
  • Cem Akin
  • Dean D. Metcalfe
  • Hans-Peter Horny
Chapter

Abstract

Mastocytosis is a unique and rare hematologic neoplasm characterized by abnormal expansion and accumulation of tissue mast cells in various organ systems. The clinical course and prognosis vary among patients, depending on the category of disease, comorbidities, and patient-related features. The cutaneous lesions of mastocytosis were first described by Nettleship and Tay in 1869. In the twentieth century, systemic forms of mastocytosis were identified and grouped into indolent and aggressive (advanced) variants. Between 1990 and 2000, diagnostic criteria and a classification of mastocytosis were established by an EU/US consensus group, and this classification was adopted by the World Health Organization (WHO) in 2001. In 2002, the European Competence Network on Mastocytosis (ECNM) was inaugurated and started. Expert representatives of this network and the consensus group served as major participants and advocates of mastocytosis research in Europe and in the USA over the past 20 years. Their achievements and the resulting clinical concepts and guidelines are summarized in the present article.

Keywords

Mastocytosis Mast cell leukemia Tryptase KIT ECNM Prognostication 

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Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  • Peter Valent
    • 1
    • 2
    Email author
  • Michel Arock
    • 3
  • Emir Hadzijusufovic
    • 1
    • 2
    • 4
  • Wolfgang R. Sperr
    • 1
    • 2
  • Karin Hartmann
    • 5
  • Cem Akin
    • 6
  • Dean D. Metcalfe
    • 7
  • Hans-Peter Horny
    • 8
  1. 1.Department of Internal Medicine I, Division of Hematology & HemostaseologyMedical University of ViennaViennaAustria
  2. 2.Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of ViennaViennaAustria
  3. 3.Cellular and Molecular Oncology, LBPA CNRS UMR8113, Ecole Normale Superiéure de Paris SaclayCachanFrance
  4. 4.Department/Clinic for Companion Animals and Horses, Clinic for Small Animals, Clinical Unit of Internal MedicineUniversity of Veterinary Medicine ViennaViennaAustria
  5. 5.Division of Allergy, Department of DermatologyUniversity of BaselBaselSwitzerland
  6. 6.Professor of Medicine, Division of Allergy and Clinical ImmunologyUniversity of MichiganAnn ArborUSA
  7. 7.Laboratory of Allergic Diseases, NIAID, NIHBethesdaUSA
  8. 8.Institute of Pathology, Ludwig Maximilian UniversityMunichGermany

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