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Erschienen in: memo - Magazine of European Medical Oncology 2/2013

01.06.2013 | short review

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


verfasst von: Peter Valent, MD, Elisabeth Aberer, MD, Christine Beham-Schmid, MD, Christina Fellinger, MD, Wolfgang Fuchs, MD, Karoline V. Gleixner, MD, Rosemarie Greul, MD, Emir Hadzijusufovic, MD, Gregor Hoermann, MD, Wolfgang R. Sperr, MD, Friedrich Wimazal, MD, Stefan Wöhrl, MD, Brigitte Zahel, MD, Hubert Pehamberger, MD

Erschienen in: memo - Magazine of European Medical Oncology | Ausgabe 2/2013

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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.
Literatur
1.
Zurück zum Zitat Parwaresch MR, Horny HP, Lennert K. Tissue mast cells in health and disease. Pathol Res Pract. 1985;179(4–5):439–61.PubMedCrossRef Parwaresch MR, Horny HP, Lennert K. Tissue mast cells in health and disease. Pathol Res Pract. 1985;179(4–5):439–61.PubMedCrossRef
2.
Zurück zum Zitat Valent P. Biology, classification and treatment of human mastocytosis. Wien Klin Wochenschr. 1996;108(13):385–97.PubMed Valent P. Biology, classification and treatment of human mastocytosis. Wien Klin Wochenschr. 1996;108(13):385–97.PubMed
3.
Zurück zum Zitat 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.PubMedCrossRef 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.PubMedCrossRef
5.
Zurück zum Zitat 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.PubMedCrossRef 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.PubMedCrossRef
6.
Zurück zum Zitat Tefferi A, Pardanani A. Systemic mastocytosis: current concepts and treatment advances. Curr Hematol Rep. 2004;3(3):197–202.PubMed Tefferi A, Pardanani A. Systemic mastocytosis: current concepts and treatment advances. Curr Hematol Rep. 2004;3(3):197–202.PubMed
7.
Zurück zum Zitat Horny HP, Sotlar K, Valent P. Mastocytosis: state of the art. Pathobiology. 2007;74(2):121–32.PubMedCrossRef Horny HP, Sotlar K, Valent P. Mastocytosis: state of the art. Pathobiology. 2007;74(2):121–32.PubMedCrossRef
9.
Zurück zum Zitat 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.PubMedCrossRef 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.PubMedCrossRef
10.
Zurück zum Zitat Nettleship E, Tay W. Rare forms of urticaria. Brit Med J. 1869;2:323–30.CrossRef Nettleship E, Tay W. Rare forms of urticaria. Brit Med J. 1869;2:323–30.CrossRef
11.
Zurück zum Zitat Ellis JM. Urticaria pigmentosa. A report of a case with autopsy. Arch Pathol. 1949;48(5):426–9. Ellis JM. Urticaria pigmentosa. A report of a case with autopsy. Arch Pathol. 1949;48(5):426–9.
12.
Zurück zum Zitat Efrati P, Klajman A, Spitz H. Mast cell leukemia? Malignant mastocytosis with leukemia-like manifestations. Blood. 1957;12(10):869–82.PubMed Efrati P, Klajman A, Spitz H. Mast cell leukemia? Malignant mastocytosis with leukemia-like manifestations. Blood. 1957;12(10):869–82.PubMed
13.
Zurück zum Zitat Friedman BI, Will JJ, Freiman DG, Braunstein H. Tissue mast cell leukemia. Blood. 1958;13(1):70–8.PubMed Friedman BI, Will JJ, Freiman DG, Braunstein H. Tissue mast cell leukemia. Blood. 1958;13(1):70–8.PubMed
14.
Zurück zum Zitat Lennert K, Parwaresch MR. Mast cells and mast cell neoplasia: a review. Histopathology. 1979;3(5):349–65.PubMedCrossRef Lennert K, Parwaresch MR. Mast cells and mast cell neoplasia: a review. Histopathology. 1979;3(5):349–65.PubMedCrossRef
15.
Zurück zum Zitat Metcalfe DD. Classification and diagnosis of mastocytosis: current status. J Invest Dermatol. 1991;96(3S):2S–4S.CrossRef Metcalfe DD. Classification and diagnosis of mastocytosis: current status. J Invest Dermatol. 1991;96(3S):2S–4S.CrossRef
16.
Zurück zum Zitat 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.PubMedCrossRef 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.PubMedCrossRef
17.
Zurück zum Zitat 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.PubMedCrossRef 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.PubMedCrossRef
18.
Zurück zum Zitat 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.PubMedCrossRef 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.PubMedCrossRef
19.
Zurück zum Zitat 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 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
20.
Zurück zum Zitat 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.PubMedCrossRef 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.PubMedCrossRef
21.
Zurück zum Zitat Schwartz LB, Irani AM. Serum tryptase and the laboratory diagnosis of systemic mastocytosis. Hematol Oncol Clin North Am. 2000;14(3):641–57.PubMedCrossRef Schwartz LB, Irani AM. Serum tryptase and the laboratory diagnosis of systemic mastocytosis. Hematol Oncol Clin North Am. 2000;14(3):641–57.PubMedCrossRef
22.
Zurück zum Zitat 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.PubMedCrossRef 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.PubMedCrossRef
23.
Zurück zum Zitat Valent P, Horny HP, Escribano L, et al. Diagnostic criteria and classification of mastocytosis: a consensus proposal. Leuk Res. 2001;25(7):603–25.PubMedCrossRef Valent P, Horny HP, Escribano L, et al. Diagnostic criteria and classification of mastocytosis: a consensus proposal. Leuk Res. 2001;25(7):603–25.PubMedCrossRef
24.
Zurück zum Zitat 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. 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.
Zurück zum Zitat 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. 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.
Zurück zum Zitat 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.PubMedCrossRef 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.PubMedCrossRef
27.
Zurück zum Zitat 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.PubMedCrossRef 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.PubMedCrossRef
28.
Zurück zum Zitat 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.PubMedCrossRef 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.PubMedCrossRef
29.
Zurück zum Zitat 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.PubMedCrossRef 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.PubMedCrossRef
30.
Zurück zum Zitat Sperr WR, Valent P. Diagnosis, progression patterns and prognostication in mastocytosis. Expert Rev Hematol. 2012;5(3):261–74.PubMedCrossRef Sperr WR, Valent P. Diagnosis, progression patterns and prognostication in mastocytosis. Expert Rev Hematol. 2012;5(3):261–74.PubMedCrossRef
31.
Zurück zum Zitat Escribano L, Akin C, Castells M, Orfao A, Metcalfe DD. Mastocytosis: current concepts in diagnosis and treatment. Ann Hematol. 2002;81(12):677–90.PubMedCrossRef Escribano L, Akin C, Castells M, Orfao A, Metcalfe DD. Mastocytosis: current concepts in diagnosis and treatment. Ann Hematol. 2002;81(12):677–90.PubMedCrossRef
32.
Zurück zum Zitat 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.PubMedCrossRef 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.PubMedCrossRef
33.
Zurück zum Zitat 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.PubMedCrossRef 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.PubMedCrossRef
34.
Zurück zum Zitat 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.PubMedCrossRef 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.PubMedCrossRef
36.
Zurück zum Zitat Akin C, Valent P, Metcalfe DD. Mast cell activation syndrome: Proposed diagnostic criteria. J Allergy Clin Immunol. 2010 Dec;126(6):1099–104.e4.PubMedCrossRef Akin C, Valent P, Metcalfe DD. Mast cell activation syndrome: Proposed diagnostic criteria. J Allergy Clin Immunol. 2010 Dec;126(6):1099–104.e4.PubMedCrossRef
37.
Zurück zum Zitat 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.PubMedCrossRef 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.PubMedCrossRef
38.
Zurück zum Zitat Valent P, Arock M, Bischoff SC, et al. The European Competence Network on Mastocytosis (ECNM). Wien Klin Wochenschr. 2004;116(19–20):647–51.PubMedCrossRef Valent P, Arock M, Bischoff SC, et al. The European Competence Network on Mastocytosis (ECNM). Wien Klin Wochenschr. 2004;116(19–20):647–51.PubMedCrossRef
39.
Zurück zum Zitat 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.PubMedCrossRef 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.PubMedCrossRef
Metadaten
Titel
The Austrian Competence Network on Mastocytosis (AUCNM): a partner and part of the European ECNM network

verfasst von
Peter Valent, MD
Elisabeth Aberer, MD
Christine Beham-Schmid, MD
Christina Fellinger, MD
Wolfgang Fuchs, MD
Karoline V. Gleixner, MD
Rosemarie Greul, MD
Emir Hadzijusufovic, MD
Gregor Hoermann, MD
Wolfgang R. Sperr, MD
Friedrich Wimazal, MD
Stefan Wöhrl, MD
Brigitte Zahel, MD
Hubert Pehamberger, MD
Publikationsdatum
01.06.2013
Verlag
Springer Vienna
Erschienen in
memo - Magazine of European Medical Oncology / Ausgabe 2/2013
Print ISSN: 1865-5041
Elektronische ISSN: 1865-5076
DOI
https://doi.org/10.1007/s12254-013-0083-y

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