Updated Diagnostic Criteria and Classification of Mast Cell Disorders: A Consensus Proposal
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 prognostica...
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| Format: | Article |
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Wiley
2021-11-01
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| Series: | HemaSphere |
| Online Access: | http://journals.lww.com/10.1097/HS9.0000000000000646 |
| _version_ | 1797285354279010304 |
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| author | 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 |
| author_facet | 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 |
| author_sort | Peter Valent |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-03-07T18:01:55Z |
| format | Article |
| id | doaj.art-baa6c70f9bed4d09abd7657782291f35 |
| institution | Directory Open Access Journal |
| issn | 2572-9241 |
| language | English |
| last_indexed | 2024-03-07T18:01:55Z |
| publishDate | 2021-11-01 |
| publisher | Wiley |
| record_format | Article |
| series | HemaSphere |
| spelling | doaj.art-baa6c70f9bed4d09abd7657782291f352024-03-02T10:44:55ZengWileyHemaSphere2572-92412021-11-01511e64610.1097/HS9.0000000000000646202111000-00003Updated Diagnostic Criteria and Classification of Mast Cell Disorders: A Consensus ProposalPeter Valent0Cem Akin1Karin Hartmann2Ivan Alvarez-Twose3Knut Brockow4Olivier Hermine5Marek Niedoszytko6Juliana Schwaab7Jonathan J. Lyons8Melody C. Carter9Hanneke Oude Elberink10Joseph H. Butterfield11Tracy I. George12Georg Greiner13Celalettin Ustun14Patrizia Bonadonna15Karl Sotlar16Gunnar Nilsson17Mohamad Jawhar18Frank Siebenhaar19Sigurd Broesby-Olsen20Selim Yavuz21Roberta Zanotti22Magdalena Lange23Boguslaw Nedoszytko24Gregor Hoermann25Mariana Castells26Deepti H. Radia27Javier I. Muñoz-Gonzalez28Wolfgang R. Sperr29Massimo Triggiani30Hanneke C. Kluin-Nelemans31Stephen J. Galli32Lawrence B. Schwartz33Andreas Reiter34Alberto Orfao35Jason Gotlib36Michel Arock37Hans-Peter Horny38Dean D. Metcalfe391 Division of Hematology and Hemostaseology, Department of Internal Medicine I, Medical University of Vienna, Austria3 Division of Allergy and Clinical Immunology, University of Michigan, Ann Arbor, Michigan, USA4 Division of Allergy, Department of Dermatology, University Hospital Basel and University of Basel, Switzerland6 Instituto de Estudios de Mastocitosis de Castilla La Mancha (CLMast) and CIBERONC, Hospital Virgen del Valle, Toledo, Spain7 Department of Dermatology and Allergy Biederstein, Technical University of Munich, Germany8 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, France9 Department of Allergology, Medical University of Gdansk, Poland10 Department of Hematology and Oncology, University Hospital Mannheim, Germany11 Translational Allergic Immunopathology Unit, Laboratory of Allergic Diseases, NIAID, NIH, Bethesda, Maryland, USA12 Mast Cell Biology Section, Laboratory of Allergic Diseases, NIAID, NIH, Bethesda, Maryland, USA13 Department of Allergology, University Medical Center Groningen and GRIAC Research Institute, University of Groningen, The Netherlands14 Division of Allergic Diseases, Mayo Clinic, Rochester, Minnesota, USA15 Department of Pathology, University of Utah, Salt Lake City, Utah, USA2 Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Austria17 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, USA18 Allergy Unit, Verona University Hospital, Verona, Italy19 Institute of Pathology, Paracelsus Medical University Salzburg, Austria20 Department of Medicine Solna & Mastocytosis Centre, Karolinska Institute and Karolinska University Hospital, Stockholm, Sweden10 Department of Hematology and Oncology, University Hospital Mannheim, Germany21 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, Germany22 Department of Dermatology and Allergy Centre, Odense University Hospital, Odense, Denmark23 Division of Haematology, Department of Internal Medicine, University of Istanbul, Turkey24 Section of Hematology, Multidisciplinary Outpatients Clinics for Mastocytosis, Department of Medicine, University Hospital of Verona, Italy25 Department of Dermatology, Venereology and Allergology, Medical University of Gdansk, Poland25 Department of Dermatology, Venereology and Allergology, Medical University of Gdansk, Poland2 Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Austria28 Division of Allergy and Immunology, Department of Medicine, Brigham and Women’s Hospital, Harvard Medical School, Boston, Massachusetts, USA29 Department of Clinical Haematology, Guys and St Thomas’ NHS Hospitals, London, United Kingdom30 Servicio Central de Citometria, Centro de Investigacion del Cancer (IBMCC; CSIC/USAL) Instituto Biosanitario de Salamanca (IBSAL) and Department of Medicine, University of Salamanca, Spain1 Division of Hematology and Hemostaseology, Department of Internal Medicine I, Medical University of Vienna, Austria31 Division of Allergy and Clinical Immunology, University of Salerno, Italy32 Department of Haematology, University Medical Center Groningen, University of Groningen, The Netherlands33 Department of Pathology and the Sean N. Parker Center for Allergy and Asthma Research, Stanford University School of Medicine, Stanford, California, USA34 Division of Rheumatology, Allergy & Immunology, Department of Internal Medicine, Virginia Commonwealth University (VCU), Richmond, Virginia, USA10 Department of Hematology and Oncology, University Hospital Mannheim, Germany30 Servicio Central de Citometria, Centro de Investigacion del Cancer (IBMCC; CSIC/USAL) Instituto Biosanitario de Salamanca (IBSAL) and Department of Medicine, University of Salamanca, Spain35 Stanford Cancer Institute/Stanford University School of Medicine/Stanford Cancer Institute, Stanford, California, USA36 Department of Hematological Biology, Pitié-Salpêtrière Hospital, Pierre et Marie Curie University (UPMC), Paris, France19 Institute of Pathology, Paracelsus Medical University Salzburg, Austria12 Mast Cell Biology Section, Laboratory of Allergic Diseases, NIAID, NIH, Bethesda, Maryland, USAMastocytosis 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.http://journals.lww.com/10.1097/HS9.0000000000000646 |
| spellingShingle | 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 Updated Diagnostic Criteria and Classification of Mast Cell Disorders: A Consensus Proposal HemaSphere |
| title | Updated Diagnostic Criteria and Classification of Mast Cell Disorders: A Consensus Proposal |
| title_full | Updated Diagnostic Criteria and Classification of Mast Cell Disorders: A Consensus Proposal |
| title_fullStr | Updated Diagnostic Criteria and Classification of Mast Cell Disorders: A Consensus Proposal |
| title_full_unstemmed | Updated Diagnostic Criteria and Classification of Mast Cell Disorders: A Consensus Proposal |
| title_short | Updated Diagnostic Criteria and Classification of Mast Cell Disorders: A Consensus Proposal |
| title_sort | updated diagnostic criteria and classification of mast cell disorders a consensus proposal |
| url | http://journals.lww.com/10.1097/HS9.0000000000000646 |
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