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Definitions, Criteria and Global Classification of Mast Cell Disorders: A Consensus Proposal

Discussion in 'Mast Cell Disorders/Mastocytosis' started by camas, Jan 12, 2013.

  1. camas

    camas Senior Member

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    Oregon
    Int Arch Allergy Immunol 2012;157:215-225 (DOI: 10.1159/000328760) Full paper here.

    Position Paper

    Definitions, Criteria and Global Classification of Mast Cell Disorders with Special Reference to Mast Cell Activation Syndromes: A Consensus Proposal

    Peter Valent, Cem Akin, Michel Arock, Knut Brockow, Joseph H. Butterfield, Melody C. Carter, Mariana Castells, Luis Escribano, Karin Hartmann, Philip Lieberman, Boguslaw Nedoszytko, Alberto Orfao, Lawrence B. Schwartz, Karl Sotlar, Wolfgang R. Sperr, Massimo Triggiani, Rudolf Valenta, Hans-Peter Horny, Dean D. Metcalfe

    Activation of tissue mast cells (MCs) and their abnormal growth and accumulation in various organs are typically found in primary MC disorders also referred to as mastocytosis. However, increasing numbers of patients are now being informed that their clinical findings are due to MC activation (MCA) that is neither associated with mastocytosis nor with a defined allergic or inflammatory reaction. In other patients with MCA, MCs appear to be clonal cells, but criteria for diagnosing mastocytosis are not met. A working conference was organized in 2010 with the aim to define criteria for diagnosing MCA and related disorders, and to propose a global unifying classification of all MC disorders and pathologic MC reactions. This classification includes three types of ‘MCA syndromes’ (MCASs), namely primary MCAS, secondary MCAS and idiopathic MCAS. MCA is now defined by robust and generally applicable criteria, including (1) typical clinical symptoms, (2) a substantial transient increase in serum total tryptase level or an increase in other MC-derived mediators, such as histamine or prostaglandin D2, or their urinary metabolites, and (3) a response of clinical symptoms to agents that attenuate the production or activities of MC mediators. These criteria should assist in the identification and diagnosis of patients with MCAS, and in avoiding misdiagnoses or overinterpretation of clinical symptoms in daily practice. Moreover, the MCAS concept should stimulate research in order to identify and exploit new molecular mechanisms and therapeutic targets.
     
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  2. MishMash

    MishMash *****

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    I'm convinced Mast Cells play a big role in our symptoms.

    BTW I attempted to help myself, five years ago, by undergoing "desensitization injections" when I was living in Hawaii.
    The injection sites on my arm just kept getting bigger and bigger. The welt kept getting redder and more painful. I never experienced any desenitization.

    In fact, my body became more acutely, alarmingly reactive to pollens, dander, dust and all the usual contaminants. It is very hard to spend any time outdoors now. I suspect my overload of Mast Cells were at fault. Unlike what the allergist said, not all allergies are mediated simply by IgE or eosinophils. But then again allergists are a very simple-minded bunch. I doubt this doc even knew what MC were.

    Has anybody else tried traditional desentizations shots? If you haven't, I would definitely not recommend them.
     
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  3. camas

    camas Senior Member

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    Hi MishMash,

    One can only hope that this paper will better educate allergists and immunologists about MCAS. There was a little controversy among patients about so much emphasis being placed on an elevated tryptase level, but they say none of this is written in stone and will evolve over time.

    Decades ago, when I first fell ill, I had the skin prick testing and reacted to nothing. The allergist just couldn't believe it because I had all the signs of bad allergies. He had me come back a few weeks later for retesting, and I reacted to everything. I'm assuming this had to do with the histamine levels in my skin?

    Anyway, I did the shots for a couple of years, but of course saw no relief because I wasn't truly allergic to anything. I've had additional allergy testing over the years with similar odd results, but have always declined any more shots.
     

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