HemaSphere (Nov 2021)
Updated Diagnostic Criteria and Classification of Mast Cell Disorders: A Consensus Proposal
- Peter Valent,
- Cem Akin,
- Karin Hartmann,
- Ivan Alvarez-Twose,
- Knut Brockow,
- Olivier Hermine,
- Marek Niedoszytko,
- Juliana Schwaab,
- Jonathan J. Lyons,
- Melody C. Carter,
- Hanneke Oude Elberink,
- Joseph H. Butterfield,
- Tracy I. George,
- Georg Greiner,
- Celalettin Ustun,
- Patrizia Bonadonna,
- Karl Sotlar,
- Gunnar Nilsson,
- Mohamad Jawhar,
- Frank Siebenhaar,
- Sigurd Broesby-Olsen,
- Selim Yavuz,
- Roberta Zanotti,
- Magdalena Lange,
- Boguslaw Nedoszytko,
- Gregor Hoermann,
- Mariana Castells,
- Deepti H. Radia,
- Javier I. Muñoz-Gonzalez,
- Wolfgang R. Sperr,
- Massimo Triggiani,
- Hanneke C. Kluin-Nelemans,
- Stephen J. Galli,
- Lawrence B. Schwartz,
- Andreas Reiter,
- Alberto Orfao,
- Jason Gotlib,
- Michel Arock,
- Hans-Peter Horny,
- Dean D. Metcalfe
Affiliations
- Peter Valent
- 1 Division of Hematology and Hemostaseology, Department of Internal Medicine I, Medical University of Vienna, Austria
- Cem Akin
- 3 Division of Allergy and Clinical Immunology, University of Michigan, Ann Arbor, Michigan, USA
- Karin Hartmann
- 4 Division of Allergy, Department of Dermatology, University Hospital Basel and University of Basel, Switzerland
- Ivan Alvarez-Twose
- 6 Instituto de Estudios de Mastocitosis de Castilla La Mancha (CLMast) and CIBERONC, Hospital Virgen del Valle, Toledo, Spain
- Knut Brockow
- 7 Department of Dermatology and Allergy Biederstein, Technical University of Munich, Germany
- Olivier Hermine
- 8 Imagine Institute Université de Paris, Sorbonne, Institut national de la santé et de la recherche médicale U1163, Centre national de référence des mastocytoses, Hôpital Necker, Assistance publique hôpitaux de Paris, France
- Marek Niedoszytko
- 9 Department of Allergology, Medical University of Gdansk, Poland
- Juliana Schwaab
- 10 Department of Hematology and Oncology, University Hospital Mannheim, Germany
- Jonathan J. Lyons
- 11 Translational Allergic Immunopathology Unit, Laboratory of Allergic Diseases, NIAID, NIH, Bethesda, Maryland, USA
- Melody C. Carter
- 12 Mast Cell Biology Section, Laboratory of Allergic Diseases, NIAID, NIH, Bethesda, Maryland, USA
- Hanneke Oude Elberink
- 13 Department of Allergology, University Medical Center Groningen and GRIAC Research Institute, University of Groningen, The Netherlands
- Joseph H. Butterfield
- 14 Division of Allergic Diseases, Mayo Clinic, Rochester, Minnesota, USA
- Tracy I. George
- 15 Department of Pathology, University of Utah, Salt Lake City, Utah, USA
- Georg Greiner
- 2 Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Austria
- Celalettin Ustun
- 17 Division of Hematology, Oncology and Cell Therapy, Department of Medicine, The Coleman Foundation Blood and Marrow Transplant Center at Rush University Medical Center, Chicago, Illinois, USA
- Patrizia Bonadonna
- 18 Allergy Unit, Verona University Hospital, Verona, Italy
- Karl Sotlar
- 19 Institute of Pathology, Paracelsus Medical University Salzburg, Austria
- Gunnar Nilsson
- 20 Department of Medicine Solna & Mastocytosis Centre, Karolinska Institute and Karolinska University Hospital, Stockholm, Sweden
- Mohamad Jawhar
- 10 Department of Hematology and Oncology, University Hospital Mannheim, Germany
- Frank Siebenhaar
- 21 Dermatological Allergology, Department of Dermatology and Allergy, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt - Universität zu Berlin, and Berlin Institute of Health, Germany
- Sigurd Broesby-Olsen
- 22 Department of Dermatology and Allergy Centre, Odense University Hospital, Odense, Denmark
- Selim Yavuz
- 23 Division of Haematology, Department of Internal Medicine, University of Istanbul, Turkey
- Roberta Zanotti
- 24 Section of Hematology, Multidisciplinary Outpatients Clinics for Mastocytosis, Department of Medicine, University Hospital of Verona, Italy
- Magdalena Lange
- 25 Department of Dermatology, Venereology and Allergology, Medical University of Gdansk, Poland
- Boguslaw Nedoszytko
- 25 Department of Dermatology, Venereology and Allergology, Medical University of Gdansk, Poland
- Gregor Hoermann
- 2 Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Austria
- Mariana Castells
- 28 Division of Allergy and Immunology, Department of Medicine, Brigham and Women’s Hospital, Harvard Medical School, Boston, Massachusetts, USA
- Deepti H. Radia
- 29 Department of Clinical Haematology, Guys and St Thomas’ NHS Hospitals, London, United Kingdom
- Javier I. Muñoz-Gonzalez
- 30 Servicio Central de Citometria, Centro de Investigacion del Cancer (IBMCC; CSIC/USAL) Instituto Biosanitario de Salamanca (IBSAL) and Department of Medicine, University of Salamanca, Spain
- Wolfgang R. Sperr
- 1 Division of Hematology and Hemostaseology, Department of Internal Medicine I, Medical University of Vienna, Austria
- Massimo Triggiani
- 31 Division of Allergy and Clinical Immunology, University of Salerno, Italy
- Hanneke C. Kluin-Nelemans
- 32 Department of Haematology, University Medical Center Groningen, University of Groningen, The Netherlands
- Stephen J. Galli
- 33 Department of Pathology and the Sean N. Parker Center for Allergy and Asthma Research, Stanford University School of Medicine, Stanford, California, USA
- Lawrence B. Schwartz
- 34 Division of Rheumatology, Allergy & Immunology, Department of Internal Medicine, Virginia Commonwealth University (VCU), Richmond, Virginia, USA
- Andreas Reiter
- 10 Department of Hematology and Oncology, University Hospital Mannheim, Germany
- Alberto Orfao
- 30 Servicio Central de Citometria, Centro de Investigacion del Cancer (IBMCC; CSIC/USAL) Instituto Biosanitario de Salamanca (IBSAL) and Department of Medicine, University of Salamanca, Spain
- Jason Gotlib
- 35 Stanford Cancer Institute/Stanford University School of Medicine/Stanford Cancer Institute, Stanford, California, USA
- Michel Arock
- 36 Department of Hematological Biology, Pitié-Salpêtrière Hospital, Pierre et Marie Curie University (UPMC), Paris, France
- Hans-Peter Horny
- 19 Institute of Pathology, Paracelsus Medical University Salzburg, Austria
- Dean D. Metcalfe
- 12 Mast Cell Biology Section, Laboratory of Allergic Diseases, NIAID, NIH, Bethesda, Maryland, USA
- DOI
- https://doi.org/10.1097/HS9.0000000000000646
- Journal volume & issue
-
Vol. 5,
no. 11
p. e646
Abstract
Mastocytosis is a hematologic neoplasm characterized by expansion and focal accumulation of neoplastic mast cells (MC) in diverse organs, including the skin, bone marrow (BM), spleen, liver, and gastrointestinal tract. The World Health Organization classification divides the disease into prognostically distinct variants of cutaneous mastocytosis (CM) and systemic mastocytosis (SM). Although this classification remains valid, recent developments in the field and the advent of new diagnostic and prognostic parameters created a need to update and refine definitions and diagnostic criteria in MC neoplasms. In addition, MC activation syndromes (MCAS) and genetic features predisposing to SM and MCAS have been identified. To discuss these developments and refinements in the classification, we organized a Working Conference comprised of experts from Europe and the United States in August 2020. This article reports on outcomes from this conference. Of particular note, we propose adjustments in the classification of CM and SM, refinements in diagnostic criteria of SM variants, including smoldering SM and BM mastocytosis (BMM), and updated criteria for MCAS and other conditions involving MC. CD30 expression in MC now qualifies as a minor SM criterion, and BMM is now defined by SM criteria, absence of skin lesions and absence of B- and C-findings. A basal serum tryptase level exceeding 20 ng/mL remains a minor SM criterion, with recognition that hereditary alpha-tryptasemia and various myeloid neoplasms may also cause elevations in tryptase. Our updated proposal will support diagnostic evaluations and prognostication in daily practice and the conduct of clinical trials in MC disorders.
