Updated Diagnostic Criteria and Classification of Mast Cell Disorders: A Consensus Proposal.


Journal

HemaSphere
ISSN: 2572-9241
Titre abrégé: Hemasphere
Pays: United States
ID NLM: 101740619

Informations de publication

Date de publication:
Nov 2021
Historique:
received: 03 08 2021
accepted: 06 09 2021
entrez: 13 12 2021
pubmed: 14 12 2021
medline: 14 12 2021
Statut: epublish

Résumé

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.

Identifiants

pubmed: 34901755
doi: 10.1097/HS9.0000000000000646
pmc: PMC8659997
doi:

Types de publication

Journal Article Review

Langues

eng

Pagination

e646

Informations de copyright

Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the European Hematology Association.

Références

Allergy. 2013 Apr;68(4):417-24
pubmed: 23409940
J Allergy Clin Immunol. 2008 Feb;121(2):519-26
pubmed: 18177694
Oncotarget. 2016 Oct 4;7(40):66299-66309
pubmed: 27602777
Histopathology. 2013 Dec;63(6):780-7
pubmed: 24111625
Hematol Oncol Clin North Am. 2011 Oct;25(5):1067-83, vii
pubmed: 22054735
Wien Klin Wochenschr. 2012 Dec;124(23-24):807-14
pubmed: 23179435
Leuk Res. 2004 Mar;28(3):249-57
pubmed: 14687620
J Allergy Clin Immunol. 2013 Nov;132(5):1234-1237.e7
pubmed: 23910691
Lancet Haematol. 2019 Dec;6(12):e638-e649
pubmed: 31676322
Am J Hematol. 2016 Sep;91(9):888-93
pubmed: 27214377
Bone. 2017 Dec;105:219-225
pubmed: 28919366
Br J Haematol. 2001 May;113(2):357-64
pubmed: 11380399
Leukemia. 2016 Dec;30(12):2342-2350
pubmed: 27416984
Leukemia. 2015 Jun;29(6):1223-32
pubmed: 25650093
Leukemia. 2016 Aug;30(8):1753-6
pubmed: 26876592
Leuk Res. 2001 Jul;25(7):603-25
pubmed: 11377686
Ann Hematol. 2014 Jan;93(1):81-8
pubmed: 24281161
Leuk Res. 2003 Jul;27(7):635-41
pubmed: 12681363
Pathobiology. 2007;74(2):121-32
pubmed: 17587883
Blood. 2010 Dec 23;116(26):5812-7
pubmed: 20855864
Ann Hematol. 2019 Dec;98(12):2693-2701
pubmed: 31686155
Expert Rev Hematol. 2014 Aug;7(4):431-7
pubmed: 25025369
J Allergy Clin Immunol. 2021 Jun;147(6):2043-2052
pubmed: 33745886
Leukemia. 2019 May;33(5):1124-1134
pubmed: 30635631
Arch Intern Med. 1966 Oct;118(4):358-62
pubmed: 4162687
J Allergy Clin Immunol. 2010 Dec;126(6):1099-104.e4
pubmed: 21035176
J Allergy Clin Immunol. 2021 Feb;147(2):622-632
pubmed: 32717252
Immunol Allergy Clin North Am. 2018 Aug;38(3):483-495
pubmed: 30007465
Front Oncol. 2020 Feb 28;10:219
pubmed: 32181155
J Allergy Clin Immunol. 2008 Jan;121(1):256-7
pubmed: 18206512
J Allergy Clin Immunol. 2014 Feb;133(2):520-8
pubmed: 23921094
J Allergy Clin Immunol Pract. 2013 Sep-Oct;1(5):474-8
pubmed: 24565619
Int J Mol Sci. 2021 Jan 02;22(1):
pubmed: 33401724
Blood. 2015 Dec 24;126(26):2832-41
pubmed: 26486787
Ann Oncol. 2014 Sep;25(9):1691-1700
pubmed: 24675021
Allergy. 2014 Oct;69(10):1267-74
pubmed: 24836395
Int Arch Allergy Immunol. 2002 Jun;128(2):136-41
pubmed: 12065914
N Engl J Med. 2015 Jul 9;373(2):163-72
pubmed: 26154789
Clin Exp Allergy. 2020 Jun;50(6):654-661
pubmed: 32107810
Nat Genet. 1996 Mar;12(3):312-4
pubmed: 8589724
Cancer. 1988 May 15;61(10):2089-94
pubmed: 2452007
J Clin Oncol. 2019 Nov 1;37(31):2846-2856
pubmed: 31509472
J Allergy Clin Immunol Pract. 2019 Apr;7(4):1125-1133.e1
pubmed: 30737190
J Allergy Clin Immunol. 2016 Jan;137(1):35-45
pubmed: 26476479
Immunol Allergy Clin North Am. 2014 May;34(2):365-81
pubmed: 24745680
J Invest Dermatol. 2010 Mar;130(3):804-15
pubmed: 19865100
Blood. 2019 Aug 1;134(5):456-468
pubmed: 31151985
Am J Surg Pathol. 2003 Jul;27(7):1013-9
pubmed: 12826896
J Clin Oncol. 2013 Feb 20;31(6):e90-7
pubmed: 23129735
Oncotarget. 2016 Jul 19;8(40):68950-68963
pubmed: 28978170
PLoS One. 2012;7(8):e43090
pubmed: 22905207
Int Arch Allergy Immunol. 2012;157(3):215-25
pubmed: 22041891
J Allergy Clin Immunol. 2016 Jan;137(1):168-178.e1
pubmed: 26100086
Medicine (Baltimore). 2016 Oct;95(41):e4934
pubmed: 27741105
Haematologica. 2011 Mar;96(3):459-63
pubmed: 21134978
Eur J Clin Invest. 2007 Jun;37(6):435-53
pubmed: 17537151
Blood. 2021 Jan 14;137(2):238-247
pubmed: 32777817
J Clin Pathol. 2004 Jun;57(6):604-8
pubmed: 15166264
J Allergy Clin Immunol. 2013 Feb;131(2):614-5
pubmed: 23273956
Mol Oncol. 2010 Aug;4(4):335-46
pubmed: 20471335
Mod Pathol. 2011 Apr;24(4):585-95
pubmed: 21186345
Immunol Allergy Clin North Am. 2014 May;34(2):383-96
pubmed: 24745681
J Clin Pathol. 1986 Jun;39(6):596-602
pubmed: 3088063
Leukemia. 2022 Feb;36(2):516-524
pubmed: 34545185
Allergy. 2014 Jun;69(6):810-3
pubmed: 24750133
J Allergy Clin Immunol. 2015 Jul;136(1):135-9
pubmed: 25605272
Int J Mol Sci. 2019 Jun 18;20(12):
pubmed: 31216696
Proc Natl Acad Sci U S A. 1995 Nov 7;92(23):10560-4
pubmed: 7479840
PLoS One. 2014 Jan 21;9(1):e85362
pubmed: 24465546
Immunol Allergy Clin North Am. 2014 May;34(2):207-18
pubmed: 24745670
Int Arch Allergy Immunol. 1999 Sep;120(1):1-7
pubmed: 10529583
Theranostics. 2021 Jan 1;11(1):292-303
pubmed: 33391475
Leukemia. 2016 Jan;30(1):136-43
pubmed: 26464169
Pathologica. 2015 Mar;107(1):14-8
pubmed: 26591626
Blood. 2004 Apr 15;103(8):3222-5
pubmed: 15070706
J Allergy Clin Immunol Pract. 2019 Sep - Oct;7(7):2387-2395.e3
pubmed: 30954641
J Clin Pathol. 2004 Mar;57(3):324-8
pubmed: 14990611
N Engl J Med. 2016 Jun 30;374(26):2530-41
pubmed: 27355533
Blood. 2006 Oct 1;108(7):2366-72
pubmed: 16741248
Curr Opin Allergy Clin Immunol. 2010 Aug;10(4):347-53
pubmed: 20485157
N Engl J Med. 1987 Jun 25;316(26):1622-6
pubmed: 3295549
Clin Cancer Res. 2005 Oct 1;11(19 Pt 1):6787-92
pubmed: 16203765
J Allergy Clin Immunol. 2009 Mar;123(3):680-6
pubmed: 19135713
J Invest Dermatol. 2021 Jul;141(7):1719-1727
pubmed: 33581142
Blood. 2013 Oct 3;122(14):2460-6
pubmed: 23958953
Blood. 2017 Mar 16;129(11):1420-1427
pubmed: 28031180
Hematol Oncol Clin North Am. 2000 Jun;14(3):641-57
pubmed: 10909044
Blood. 2008 Aug 15;112(4):946-56
pubmed: 18684881
Lancet Haematol. 2021 Mar;8(3):e194-e204
pubmed: 33508247
J Allergy Clin Immunol. 2015 Dec;136(6):1673-1679.e3
pubmed: 26044856
Blood. 2020 Apr 16;135(16):1365-1376
pubmed: 32106312
Nat Genet. 2016 Dec;48(12):1564-1569
pubmed: 27749843
Expert Rev Hematol. 2014 Oct;7(5):683-90
pubmed: 25169217
Leuk Lymphoma. 2011 May;52(5):740-4
pubmed: 21261503
Ann Oncol. 2017 Oct 1;28(10):2367-2376
pubmed: 28945834
Blood. 2009 Jun 4;113(23):5727-36
pubmed: 19363219
Cancer Res. 2017 Mar 15;77(6):1261-1270
pubmed: 28254862
Genes Chromosomes Cancer. 2018 May;57(5):252-259
pubmed: 29341334
Int Arch Allergy Immunol. 2012;157(4):399-405
pubmed: 22123213
Int J Mol Sci. 2020 Nov 27;21(23):
pubmed: 33261124
Haematologica. 2017 Jun;102(6):1035-1043
pubmed: 28255023
Osteoporos Int. 2016 Aug;27(8):2411-21
pubmed: 26892042
Int Arch Allergy Immunol. 2002 Feb;127(2):140-2
pubmed: 11919425
J Clin Oncol. 2014 Oct 10;32(29):3264-74
pubmed: 25154823
J Allergy Clin Immunol. 2015 Dec;136(6):1581-1590.e3
pubmed: 26152315

Auteurs

Peter Valent (P)

Division of Hematology and Hemostaseology, Department of Internal Medicine I, Medical University of Vienna, Austria.
Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Austria.

Cem Akin (C)

Division of Allergy and Clinical Immunology, University of Michigan, Ann Arbor, Michigan, USA.

Karin Hartmann (K)

Division of Allergy, Department of Dermatology, University Hospital Basel and University of Basel, Switzerland.
Department of Biomedicine, University Hospital Basel and University of Basel, Switzerland.

Ivan Alvarez-Twose (I)

Instituto de Estudios de Mastocitosis de Castilla La Mancha (CLMast) and CIBERONC, Hospital Virgen del Valle, Toledo, Spain.

Knut Brockow (K)

Department of Dermatology and Allergy Biederstein, Technical University of Munich, Germany.

Olivier Hermine (O)

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 (M)

Department of Allergology, Medical University of Gdansk, Poland.

Juliana Schwaab (J)

Department of Hematology and Oncology, University Hospital Mannheim, Germany.

Jonathan J Lyons (JJ)

Translational Allergic Immunopathology Unit, Laboratory of Allergic Diseases, NIAID, NIH, Bethesda, Maryland, USA.

Melody C Carter (MC)

Mast Cell Biology Section, Laboratory of Allergic Diseases, NIAID, NIH, Bethesda, Maryland, USA.

Hanneke Oude Elberink (HO)

Department of Allergology, University Medical Center Groningen and GRIAC Research Institute, University of Groningen, The Netherlands.

Joseph H Butterfield (JH)

Division of Allergic Diseases, Mayo Clinic, Rochester, Minnesota, USA.

Tracy I George (TI)

Department of Pathology, University of Utah, Salt Lake City, Utah, USA.

Georg Greiner (G)

Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Austria.
Department of Laboratory Medicine, Medical University of Vienna, Austria and Ihr Labor, Medical Diagnostic Laboratories, Vienna, Austria.

Celalettin Ustun (C)

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 (P)

Allergy Unit, Verona University Hospital, Verona, Italy.

Karl Sotlar (K)

Institute of Pathology, Paracelsus Medical University Salzburg, Austria.

Gunnar Nilsson (G)

Department of Medicine Solna & Mastocytosis Centre, Karolinska Institute and Karolinska University Hospital, Stockholm, Sweden.

Mohamad Jawhar (M)

Department of Hematology and Oncology, University Hospital Mannheim, Germany.

Frank Siebenhaar (F)

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 (S)

Department of Dermatology and Allergy Centre, Odense University Hospital, Odense, Denmark.

Selim Yavuz (S)

Division of Haematology, Department of Internal Medicine, University of Istanbul, Turkey.

Roberta Zanotti (R)

Section of Hematology, Multidisciplinary Outpatients Clinics for Mastocytosis, Department of Medicine, University Hospital of Verona, Italy.

Magdalena Lange (M)

Department of Dermatology, Venereology and Allergology, Medical University of Gdansk, Poland.

Boguslaw Nedoszytko (B)

Department of Dermatology, Venereology and Allergology, Medical University of Gdansk, Poland.
Invicta Fertility and Reproductive Center, Molecular Laboratory, Sopot, Poland.

Gregor Hoermann (G)

Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Austria.
MLL Munich Leukemia Laboratory, Munich, Germany.

Mariana Castells (M)

Division of Allergy and Immunology, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.

Deepti H Radia (DH)

Department of Clinical Haematology, Guys and St Thomas' NHS Hospitals, London, United Kingdom.

Javier I Muñoz-Gonzalez (JI)

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 (WR)

Division of Hematology and Hemostaseology, Department of Internal Medicine I, Medical University of Vienna, Austria.
Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Austria.

Massimo Triggiani (M)

Division of Allergy and Clinical Immunology, University of Salerno, Italy.

Hanneke C Kluin-Nelemans (HC)

Department of Haematology, University Medical Center Groningen, University of Groningen, The Netherlands.

Stephen J Galli (SJ)

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 (LB)

Division of Rheumatology, Allergy & Immunology, Department of Internal Medicine, Virginia Commonwealth University (VCU), Richmond, Virginia, USA.

Andreas Reiter (A)

Department of Hematology and Oncology, University Hospital Mannheim, Germany.

Alberto Orfao (A)

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 (J)

Stanford Cancer Institute/Stanford University School of Medicine/Stanford Cancer Institute, Stanford, California, USA.

Michel Arock (M)

Department of Hematological Biology, Pitié-Salpêtrière Hospital, Pierre et Marie Curie University (UPMC), Paris, France.

Hans-Peter Horny (HP)

Institute of Pathology, Paracelsus Medical University Salzburg, Austria.
Institute of Pathology, Ludwig-Maximilians-University, Munich, Germany.

Dean D Metcalfe (DD)

Mast Cell Biology Section, Laboratory of Allergic Diseases, NIAID, NIH, Bethesda, Maryland, USA.

Classifications MeSH