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

SWAlexander

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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.
https://journals.lww.com/hemasphere...gnostic_Criteria_and_Classification_of.3.aspx
 

Inara

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I don't find it helpful that both groups, the "tryptase group" (to which this publication belongs - Valent is one of *the* tryptase proponents: it's only MCAS if you have anaphylactic shocks/elevated blood tryptase) and the "non tryptase-group", each write about "consensus criteria" if there really is no consensus. None of the "non tryptase-group" is one of the authors of this publication, as far as I can see, so I don't see a consensus. They obviously mean a "consensus" amongst each group, but not between both groups.

Tryptase group: consensus is "elevated tryptase"
Non tryptase-group: consensus criteria don't contain "elevated tryptase".

This is a new (political) level that I find very disappointing. It's still rule and divide: Only those with elevated blood tryptase are the true MCAS people.

Where's the research? We don't need opinion.

Have I misunderstood? Please correct me.
 

SWAlexander

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Inara:
It is not a scientific paper, it is an article.
Maybe it would be much more helpful if you write to HemaSphere Service hemasphere@ehaweb.org directly, since I cannot influence there publication. I am just a patient seeking information.
 
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