Updated Diagnostic Criteria and Classification of Mast Cell Disorders

Autor: Joseph H. Butterfield, Gunnar Nilsson, Melody C. Carter, Hanneke Oude Elberink, Bogusław Nedoszytko, Deepti Radia, Mariana Castells, Roberta Zanotti, Magdalena Lange, Cem Akin, Georg Greiner, Iván Álvarez-Twose, Javier I. Muñoz-González, Hanneke C. Kluin-Nelemans, Karin Hartmann, Peter Valent, Stephen J. Galli, Frank Siebenhaar, Karl Sotlar, Patrizia Bonadonna, Tracy I. George, Gregor Hoermann, Knut Brockow, Massimo Triggiani, Celalettin Ustun, Alberto Orfao, Lawrence B. Schwartz, Mohamad Jawhar, Sigurd Broesby-Olsen, Juliana Schwaab, Marek Niedoszytko, Wolfgang R. Sperr, Olivier Hermine, Andreas Reiter, Jason Gotlib, Selim Yavuz, Michel Arock, Dean D. Metcalfe, Hans-Peter Horny, Jonathan J. Lyons
Přispěvatelé: Groningen Research Institute for Asthma and COPD (GRIAC)
Jazyk: angličtina
Rok vydání: 2021
Předmět:
Zdroj: HemaSphere, 5(11):646. LIPPINCOTT WILLIAMS & WILKINS
HemaSphere, Vol 5, Iss 11, p e646 (2021)
Valent, P, Akin, C, Hartmann, K, Alvarez-Twose, I, Brockow, K, Hermine, O, Niedoszytko, M, Schwaab, J, Lyons, J J, Carter, M C, Elberink, H O, Butterfield, J H, George, T I, Greiner, G, Ustun, C, Bonadonna, P, Sotlar, K, Nilsson, G, Jawhar, M, Siebenhaar, F, Broesby-Olsen, S, Yavuz, S, Zanotti, R, Lange, M, Nedoszytko, B, Hoermann, G, Castells, M, Radia, D H, Muñoz-Gonzalez, J I, Sperr, W R, Triggiani, M, Kluin-Nelemans, H C, Galli, S J, Schwartz, L B, Reiter, A, Orfao, A, Gotlib, J, Arock, M, Horny, H-P & Metcalfe, D D 2021, ' Updated Diagnostic Criteria and Classification of Mast Cell Disorders : A Consensus Proposal ', HemaSphere, vol. 5, no. 11, e646 . https://doi.org/10.1097/HS9.0000000000000646
HemaSphere
ISSN: 2572-9241
DOI: 10.1097/HS9.0000000000000646
Popis: 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.
Databáze: OpenAIRE
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