Dataset Information

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

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.

SUBMITTER: Valent P

PROVIDER: S-EPMC8659997 | biostudies-literature | 2021 Nov

REPOSITORIES: biostudies-literature

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

Valent Peter P Akin Cem C Hartmann Karin K Alvarez-Twose Ivan I Brockow Knut K Hermine Olivier O Niedoszytko Marek M Schwaab Juliana J Lyons Jonathan J JJ Carter Melody C MC Elberink Hanneke Oude HO Butterfield Joseph H JH George Tracy I TI Greiner Georg G Ustun Celalettin C Bonadonna Patrizia P Sotlar Karl K Nilsson Gunnar G Jawhar Mohamad M Siebenhaar Frank F Broesby-Olsen Sigurd S Yavuz Selim S Zanotti Roberta R Lange Magdalena M Nedoszytko Boguslaw B Hoermann Gregor G Castells Mariana M Radia Deepti H DH Muñoz-Gonzalez Javier I JI Sperr Wolfgang R WR Triggiani Massimo M Kluin-Nelemans Hanneke C HC Galli Stephen J SJ Schwartz Lawrence B LB Reiter Andreas A Orfao Alberto A Gotlib Jason J Arock Michel M Horny Hans-Peter HP Metcalfe Dean D DD

HemaSphere 20211013 11

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 p ...[more]

PMID: 34901755

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Project description:ObjectiveTo develop evidence-informed, expert consensus research diagnostic criteria for traumatic encephalopathy syndrome (TES), the clinical disorder associated with neuropathologically diagnosed chronic traumatic encephalopathy (CTE).MethodsA panel of 20 expert clinician-scientists in neurology, neuropsychology, psychiatry, neurosurgery, and physical medicine and rehabilitation, from 11 academic institutions, participated in a modified Delphi procedure to achieve consensus, initiated at the First National Institute of Neurological Disorders and Stroke Consensus Workshop to Define the Diagnostic Criteria for TES, April, 2019. Before consensus, panelists reviewed evidence from all published cases of CTE with neuropathologic confirmation, and they examined the predictive validity data on clinical features in relation to CTE pathology from a large clinicopathologic study (n = 298).ResultsConsensus was achieved in 4 rounds of the Delphi procedure. Diagnosis of TES requires (1) substantial exposure to repetitive head impacts (RHIs) from contact sports, military service, or other causes; (2) core clinical features of cognitive impairment (in episodic memory and/or executive functioning) and/or neurobehavioral dysregulation; (3) a progressive course; and (4) that the clinical features are not fully accounted for by any other neurologic, psychiatric, or medical conditions. For those meeting criteria for TES, functional dependence is graded on 5 levels, ranging from independent to severe dementia. A provisional level of certainty for CTE pathology is determined based on specific RHI exposure thresholds, core clinical features, functional status, and additional supportive features, including delayed onset, motor signs, and psychiatric features.ConclusionsNew consensus diagnostic criteria for TES were developed with a primary goal of facilitating future CTE research. These criteria will be revised as updated clinical and pathologic information and in vivo biomarkers become available.

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

Publications

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

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