Skip to main content
SpringerLink
Log in

The Austrian Competence Network on Mastocytosis (AUCNM): a partner and part of the European ECNM network


  • short review
  • Published:
memo - Magazine of European Medical Oncology Aims and scope Submit manuscript

Abstract

Mastocytosis is a group of rare diseases characterized by abnormal expansion and accumulation of tissue mast cells in various organ systems. The disease can be divided into cutaneous and systemic variants. Although considered a rare pathologic condition, more and more patients are currently diagnosed as suffering from mastocytosis. The increasing incidence is best explained by enhanced awareness and improved diagnostics in the Western world. This has in turn created a need to establish optimal facilities for the diagnosis, management, and therapy of patients with mastocytosis. In 2002, the European Competence Network on Mastocytosis (ECNM) was established, with the aim to provide all available information to doctors and patients, and to improve management and therapy of mastocytosis in Europe. Within the ECNM, Centers of Excellence and Reference Centers have been defined and inaugurated. In addition, several countries established a local network of competence within the ECNM. In 2011, the Austrian Competence Network on Mastocytosis (AUCNM) was inaugurated. The AUCNM serves as an integral part and essential partner of the ECNM. In the current article, the structure, aims, achievements, and ongoing projects of the AUCNM are presented.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

Abbreviations

AHNMD:

Associated hematologic non-mast-cell disease

ASM:

Aggressive systemic mastocytosis

AUCNM:

Austrian Competence Network on Mastocytosis

CM:

Cutaneous mastocytosis

ECNM:

European Competence Network on Mastocytosis

ISM:

Indolent systemic mastocytosis

MCAS:

Mast cell activation syndrome

MC:

Mast cells

MCL:

Mast cell leukemia

MIS:

Mastocytosis in the skin

MPN:

Myeloproliferative neoplasm(s)

SM:

Systemic mastocytosis

SSM:

Smoldering systemic mastocytosis

TKI:

Tyrosine kinase inhibitor(s)

UP:

Urticaria pigmentosa

WHO:

World Health Organization

References

  1. Parwaresch MR, Horny HP, Lennert K. Tissue mast cells in health and disease. Pathol Res Pract. 1985;179(4–5):439–61.

    Article  PubMed  CAS  Google Scholar 

  2. Valent P. Biology, classification and treatment of human mastocytosis. Wien Klin Wochenschr. 1996;108(13):385–97.

    PubMed  CAS  Google Scholar 

  3. Valent P, Akin C, Sperr WR, et al. Diagnosis and treatment of systemic mastocytosis: state of the art. Br J Haematol. 2003;122(5):695–717.

    Article  PubMed  Google Scholar 

  4. Akin C, Metcalfe DD. Systemic mastocytosis. Annu Rev Med. 2004;55:419–32.

    Article  PubMed  CAS  Google Scholar 

  5. Valent P, Sperr WR, Schwartz LB, Horny HP. Diagnosis and classification of mast cell proliferative disorders: delineation from immunologic diseases and non-mast cell hematopoietic neoplasms. J Allergy Clin Immunol. 2004;114(1):3–11.

    Article  PubMed  CAS  Google Scholar 

  6. Tefferi A, Pardanani A. Systemic mastocytosis: current concepts and treatment advances. Curr Hematol Rep. 2004;3(3):197–202.

    PubMed  Google Scholar 

  7. Horny HP, Sotlar K, Valent P. Mastocytosis: state of the art. Pathobiology. 2007;74(2):121–32.

    Article  PubMed  CAS  Google Scholar 

  8. Metcalfe DD. Mast cells and mastocytosis. Blood. 2008;112(4):946–56.

    Article  PubMed  CAS  Google Scholar 

  9. Arock M, Valent P. Pathogenesis, classification and treatment of mastocytosis: state of the art in 2010 and future perspectives. Expert Rev Hematol. 2010;3(4):497–516.

    Article  PubMed  Google Scholar 

  10. Nettleship E, Tay W. Rare forms of urticaria. Brit Med J. 1869;2:323–30.

    Article  Google Scholar 

  11. Ellis JM. Urticaria pigmentosa. A report of a case with autopsy. Arch Pathol. 1949;48(5):426–9.

    CAS  Google Scholar 

  12. Efrati P, Klajman A, Spitz H. Mast cell leukemia? Malignant mastocytosis with leukemia-like manifestations. Blood. 1957;12(10):869–82.

    PubMed  CAS  Google Scholar 

  13. Friedman BI, Will JJ, Freiman DG, Braunstein H. Tissue mast cell leukemia. Blood. 1958;13(1):70–8.

    PubMed  CAS  Google Scholar 

  14. Lennert K, Parwaresch MR. Mast cells and mast cell neoplasia: a review. Histopathology. 1979;3(5):349–65.

    Article  PubMed  CAS  Google Scholar 

  15. Metcalfe DD. Classification and diagnosis of mastocytosis: current status. J Invest Dermatol. 1991;96(3S):2S–4S.

    Article  Google Scholar 

  16. Nagata H, Worobec AS, Oh CK, et al. Identification of a point mutation in the catalytic domain of the protooncogene c-kit in peripheral blood mononuclear cells of patients who have mastocytosis with an associated hematologic disorder. Proc Natl Acad Sci (USA). 1995;92(23):10560–4.

    Article  PubMed  CAS  Google Scholar 

  17. Longley BJ, Tyrrell L, Lu SZ, et al. Somatic c-KIT activating mutation in urticaria pigmentosa and aggressive mastocytosis: establishment of clonality in a human mast cell neoplasm. Nat Genet. 1996;12(3):312–4.

    Article  PubMed  CAS  Google Scholar 

  18. Fritsche-Polanz R, Jordan JH, Feix A, et al. Mutation analysis of C-KIT in patients with myelodysplastic syndromes without mastocytosis and cases of systemic mastocytosis. Br J Haematol. 2001;113(2):357–64.

    Article  PubMed  CAS  Google Scholar 

  19. Escribano L, Orfao A, Díaz-Agustin B, et al. Indolent systemic mast cell disease in adults: immunophenotypic characterization of bone marrow mast cells and its diagnostic implications. Blood. 1998;91(8):2731–6.

    PubMed  CAS  Google Scholar 

  20. Horny HP, Sillaber C, Menke D, et al. Diagnostic value of immunostaining for tryptase in patients with mastocytosis. Am J Surg Pathol. 1998;22(9):1132–40.

    Article  PubMed  CAS  Google Scholar 

  21. Schwartz LB, Irani AM. Serum tryptase and the laboratory diagnosis of systemic mastocytosis. Hematol Oncol Clin North Am. 2000;14(3):641–57.

    Article  PubMed  CAS  Google Scholar 

  22. Sperr WR, Escribano L, Jordan JH, et al. Morphologic properties of neoplastic mast cells: delineation of stages of maturation and implication for cytological grading of mastocytosis. Leuk Res. 2001;25(7):529–36.

    Article  PubMed  CAS  Google Scholar 

  23. Valent P, Horny HP, Escribano L, et al. Diagnostic criteria and classification of mastocytosis: a consensus proposal. Leuk Res. 2001;25(7):603–25.

    Article  PubMed  CAS  Google Scholar 

  24. Valent P, Horny H-P, Li CY, Longley JB, Metcalfe DD, Parwaresch RM, Bennett JM. Mastocytosis. In: Jaffe ES, Harris NL, Stein H, Vardiman JW, editors. World Health Organization (WHO) classification of tumours of haematopoietic and lymphoid tissues. Lyon: IARC Press; 2001. pp. 291–302.

  25. Horny HP, Akin C, Metcalfe DD, Bain BJ, Akin C, Escribano L, et al. Mastocytosis (mast cell disease). In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW, editors. World Health Organization (WHO) classification of tumours. Pathology & genetics. Tumours of haematopoietic and lymphoid tissues. Lyon: IARC Press; 2008. pp. 54–63.

  26. Valent P, Akin C, Sperr WR, Horny HP, Metcalfe DD. Mast cell proliferative disorders: current view on variants recognized by the World Health Organization. Hematol Oncol Clin North Am. 2003;17(5):1227–41.

    Article  PubMed  Google Scholar 

  27. Valent P, Akin C, Sperr WR, et al. Aggressive systemic mastocytosis and related mast cell disorders: current treatment options and proposed response criteria. Leuk Res. 2003;27(7):635–41.

    Article  PubMed  CAS  Google Scholar 

  28. Lim KH, Tefferi A, Lasho TL, et al. Systemic mastocytosis in 342 consecutive adults: survival studies and prognostic factors. Blood. 2009;113(23):5727–36.

    Article  PubMed  CAS  Google Scholar 

  29. Pardanani A, Lim KH, Lasho TL, et al. WHO subvariants of indolent mastocytosis: clinical details and prognostic evaluation in 159 consecutive adults. Blood. 2010;115(1):150–51.

    Article  PubMed  CAS  Google Scholar 

  30. Sperr WR, Valent P. Diagnosis, progression patterns and prognostication in mastocytosis. Expert Rev Hematol. 2012;5(3):261–74.

    Article  PubMed  CAS  Google Scholar 

  31. Escribano L, Akin C, Castells M, Orfao A, Metcalfe DD. Mastocytosis: current concepts in diagnosis and treatment. Ann Hematol. 2002;81(12):677–90.

    Article  PubMed  CAS  Google Scholar 

  32. Valent P, Akin C, Escribano L, et al. Standards and standardization in mastocytosis: consensus statements on diagnostics, treatment recommendations and response criteria. Eur J Clin Invest. 2007;37(6):435–53.

    Article  PubMed  CAS  Google Scholar 

  33. Bonadonna P, Perbellini O, Passalacqua G, et al. Clonal mast cell disorders in patients with systemic reactions to Hymenoptera stings and increased serum tryptase levels. J Allergy Clin Immunol. 2009;123(3):680–86.

    Article  PubMed  CAS  Google Scholar 

  34. Alvarez-Twose I, González de Olano D, Sánchez-Muñoz L, et al. Clinical, biological, and molecular characteristics of clonal mast cell disorders presenting with systemic mast cell activation symptoms. J Allergy Clin Immunol. 2010;125(6):1269–1278.e2.

    Article  PubMed  CAS  Google Scholar 

  35. Brockow K, Metcalfe DD. Mastocytosis. Chem Immunol Allergy. 2010;95:110–24.

    Article  PubMed  CAS  Google Scholar 

  36. Akin C, Valent P, Metcalfe DD. Mast cell activation syndrome: Proposed diagnostic criteria. J Allergy Clin Immunol. 2010 Dec;126(6):1099–104.e4.

    Article  PubMed  CAS  Google Scholar 

  37. Valent P, Akin C, Arock M, et al. Definitions, criteria and global classification of mast cell disorders with special reference to mast cell activation syndromes: a consensus proposal. Int Arch Allergy Immunol. 2012;157(3):215–25.

    Article  PubMed  Google Scholar 

  38. Valent P, Arock M, Bischoff SC, et al. The European Competence Network on Mastocytosis (ECNM). Wien Klin Wochenschr. 2004;116(19–20):647–51.

    Article  PubMed  Google Scholar 

  39. Valent P, Arock M, Bonadonna P, et al. European Competence Network on Mastocytosis (ECNM): 10-year jubilee, update, and future perspectives. Wien Klin Wochenschr. 2012;124(23–24):807–14.

    Article  PubMed  Google Scholar 

Download references

Conflict of interest


The authors declare that there are no actual or potential conflicts of interest in relation to this article.

Author information

Authors and Affiliations

  1. Department of Medicine I, Division of Hematology & Hemostaseology, Medical University of Vienna, Währinger Gürtel 18–20, Vienna, Austria

    Peter Valent MD, Karoline V. Gleixner MD, Emir Hadzijusufovic MD & Wolfgang R. Sperr MD

  2. Ludwig Boltzmann Cluster Oncology, Medical University of Vienna, Vienna, Austria

    Peter Valent MD, Karoline V. Gleixner MD, Emir Hadzijusufovic MD & Wolfgang R. Sperr MD

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

    Elisabeth Aberer MD

  4. Institute of Pathology, Medical University of Graz, Graz, Austria

    Christine Beham-Schmid MD

  5. Floridsdorf Allergy Center (FAZ), Vienna, Austria

    Christina Fellinger MD & Stefan Wöhrl MD

  6. Department of Dermatology, General Hospital of Linz, Linz, Austria

    Wolfgang Fuchs MD & Brigitte Zahel MD

  7. Department of Oncology, General Hospital of Linz, Linz, Austria

    Rosemarie Greul MD

  8. Department for Companion Animals and Horses, Clinic for Internal Medicine and Infectious Diseases, University of Veterinary Medicine Vienna, Vienna, Austria

    Emir Hadzijusufovic MD

  9. Department of Laboratory Medicine, Medical University of Vienna, Vienna, Austria

    Gregor Hoermann MD

  10. Department of Obstetrics and Gynecology, Medical University of Vienna, Vienna, Austria

    Friedrich Wimazal MD

  11. Department of Dermatology, Medical University of Vienna, Vienna, Austria

    Stefan Wöhrl MD & Hubert Pehamberger MD

Authors
  1. Peter Valent MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Elisabeth Aberer MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Christine Beham-Schmid MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Christina Fellinger MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Wolfgang Fuchs MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Karoline V. Gleixner MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Rosemarie Greul MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Emir Hadzijusufovic MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Gregor Hoermann MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Wolfgang R. Sperr MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. Friedrich Wimazal MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  12. Stefan Wöhrl MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  13. Brigitte Zahel MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  14. Hubert Pehamberger MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Peter Valent MD.

Additional information

The AUCNM was initiated by an inauguration meeting in Vienna (General Hospital of Vienna at Medical University of Vienna) on November 25, 2011.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Valent, P., Aberer, E., Beham-Schmid, C. et al. The Austrian Competence Network on Mastocytosis (AUCNM): a partner and part of the European ECNM network
. memo 6, 114–118 (2013). https://doi.org/10.1007/s12254-013-0083-y

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12254-013-0083-y

Keywords

Search

Navigation