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  1. Article ; Online: Is lirentelimab the 'magic bullet' to fight pathological mast-cell activation in systemic mastocytosis?

    Barete, Stéphane / Arock, Michel

    The British journal of dermatology

    2023  Volume 189, Issue 5, Page(s) 503–504

    MeSH term(s) Humans ; Mastocytosis, Systemic/drug therapy ; Mast Cells ; Antineoplastic Agents ; Antibodies, Monoclonal
    Chemical Substances Antineoplastic Agents ; Antibodies, Monoclonal
    Language English
    Publishing date 2023-07-04
    Publishing country England
    Document type Editorial ; Comment
    ZDB-ID 80076-4
    ISSN 1365-2133 ; 0007-0963
    ISSN (online) 1365-2133
    ISSN 0007-0963
    DOI 10.1093/bjd/ljad227
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  2. Article ; Online: Reversible Elevation of Tryptase Over the Individual's Baseline: Why is It the Best Biomarker for Severe Systemic Mast Cell Activation and MCAS?

    Valent, Peter / Akin, Cem / Arock, Michel

    Current allergy and asthma reports

    2024  Volume 24, Issue 3, Page(s) 133–141

    Abstract: Purpose of review: Mast cell (MC) activation syndromes (MCAS) are conditions defined by recurrent episodes of severe systemic anaphylaxis or similar systemic events triggered by MC-derived mediators that can be measured in biological fluids. Since some ... ...

    Abstract Purpose of review: Mast cell (MC) activation syndromes (MCAS) are conditions defined by recurrent episodes of severe systemic anaphylaxis or similar systemic events triggered by MC-derived mediators that can be measured in biological fluids. Since some symptoms of MC activation may occur due to other, non-MC etiologies and lead to confusion over diagnosis, it is of crucial importance to document the involvement of MC and their products in the patients´ symptomatology.
    Recent findings: The most specific and generally accepted marker of severe systemic MC activation is an event-related, transient increase in the serum tryptase level over the individual baseline of the affected individual. However, baseline concentrations of serum tryptase vary among donors, depending on the genetic background, age, kidney function, and underlying disease. As a result, it is of critical importance to provide a flexible equation that defines the diagnostic increase in tryptase qualifying as MCAS criterion in all patients, all situations, and all ranges of baseline serum tryptase. In 2012, the consensus group proposed the 120% + 2 ng/ml formula, which covers the great majority of groups, including cases with low, normal, or elevated basal serum tryptase level. This formula has been validated in subsequent studies and has proven to be a robust and consistent diagnostic criterion of MCAS. The present article is discussing the impact of this formula and possible limitations as well as alternative markers and mediators that may be indicative of MCAS.
    MeSH term(s) Humans ; Mast Cells ; Mastocytosis/diagnosis ; Tryptases ; Anaphylaxis/diagnosis ; Biomarkers
    Chemical Substances Tryptases (EC 3.4.21.59) ; Biomarkers
    Language English
    Publishing date 2024-02-03
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 2057370-4
    ISSN 1534-6315 ; 1529-7322
    ISSN (online) 1534-6315
    ISSN 1529-7322
    DOI 10.1007/s11882-024-01124-2
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  3. Article ; Online: Case Report: A family history of peanut allergy and hereditary alpha-tryptasemia.

    Chantran, Yannick / Renaudin, Hélène / Arock, Michel / Guiddir, Tamazoust / Nemni, Ariane

    Frontiers in allergy

    2024  Volume 4, Page(s) 1322117

    Abstract: Context: Hereditary alpha-tryptasemia (HαT) is associated with elevated basal serum tryptase (bST) and is associated with a higher risk of severe anaphylactic reactions in patients with clonal mast cell disorders or IgE-mediated Hymenoptera venom- ... ...

    Abstract Context: Hereditary alpha-tryptasemia (HαT) is associated with elevated basal serum tryptase (bST) and is associated with a higher risk of severe anaphylactic reactions in patients with clonal mast cell disorders or IgE-mediated Hymenoptera venom-induced anaphylaxis. The consequence of this genetic trait remains to be determined in other allergic diseases and food allergy in particular.
    Objectives: Here, we describe three cases of peanut allergy among siblings from a single family of four: two of them were associated with HαT, and the third one was associated with the tryptase wild-type genotype.
    Methods: TPSAB1/TPSB2
    Results: Compared to the sibling with the conventional tryptase genotype, the two siblings with HαT presented a lower peanut threshold at the initial oral food challenge, higher peanut skin prick test reactivity, higher levels of specific IgE to peanut, Ara h 2, and Ara h 6, and a lower IgG4/IgE ratio after 10 years of oral immunotherapy.
    Conclusion: The tryptase genotype and HαT status might modify the clinical presentation and biological features of food allergy.
    Language English
    Publishing date 2024-01-23
    Publishing country Switzerland
    Document type Case Reports
    ISSN 2673-6101
    ISSN (online) 2673-6101
    DOI 10.3389/falgy.2023.1322117
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  4. Article ; Online: A new therapeutic advance for symptomatic systemic mastocytosis?

    Arock, Michel

    Lancet (London, England)

    2017  Volume 389, Issue 10069, Page(s) 576–578

    MeSH term(s) Humans ; Mastocytosis ; Mastocytosis, Systemic
    Language English
    Publishing date 2017--11
    Publishing country England
    Document type Journal Article ; Comment
    ZDB-ID 3306-6
    ISSN 1474-547X ; 0023-7507 ; 0140-6736
    ISSN (online) 1474-547X
    ISSN 0023-7507 ; 0140-6736
    DOI 10.1016/S0140-6736(16)31655-5
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  5. Article ; Online: KIT Mutations and Other Genetic Defects in Mastocytosis: Implications for Disease Pathology and Targeted Therapies.

    Chantran, Yannick / Valent, Peter / Arock, Michel

    Immunology and allergy clinics of North America

    2023  Volume 43, Issue 4, Page(s) 651–664

    Abstract: A KIT activating mutation (usually KIT D816V) is detected in neoplastic cells in greater than 90% of indolent patients with systemic mastocytosis (SM). In more advanced variants of SM, additional genetic defects can be found in several myeloid malignancy- ...

    Abstract A KIT activating mutation (usually KIT D816V) is detected in neoplastic cells in greater than 90% of indolent patients with systemic mastocytosis (SM). In more advanced variants of SM, additional genetic defects can be found in several myeloid malignancy-related genes, which can be detected by applying next-generation sequencing. Currently, the techniques recommended to detect the KIT D816V mutation and quantify the mutational burden in peripheral blood, bone marrow, or other organs/tissues are allele specific-quantitative PCR or droplet digital PCR. These techniques are useful for diagnosis, prognostication, follow-up and monitoring of therapeutic efficacy of cytoreductive agents in patients with SM.
    MeSH term(s) Humans ; Proto-Oncogene Proteins c-kit/genetics ; Mastocytosis/diagnosis ; Mastocytosis/genetics ; Mastocytosis/therapy ; Mutation ; Mastocytosis, Systemic/diagnosis ; Mastocytosis, Systemic/drug therapy ; Mastocytosis, Systemic/genetics ; Bone Marrow ; Mast Cells
    Chemical Substances Proto-Oncogene Proteins c-kit (EC 2.7.10.1)
    Language English
    Publishing date 2023-06-11
    Publishing country United States
    Document type Journal Article ; Review ; Research Support, Non-U.S. Gov't
    ZDB-ID 92606-1
    ISSN 1557-8607 ; 0889-8561
    ISSN (online) 1557-8607
    ISSN 0889-8561
    DOI 10.1016/j.iac.2023.04.008
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  6. Book ; Online: Autoformation et aide au diagnostic en hématologie avec le logiciel ADH

    Arock, Michel / Chemla, Gilbert / Chemla, Jean-Paul

    2008  

    Author's details by Michel Arock, Gilbert Chemla, Jean-Paul Chemla
    Keywords Bioinformatics ; Biology/Data processing ; Cell biology ; Hematology ; Medical laboratories ; Microscopy
    Language French
    Publisher Springer-Verlag France, Paris
    Publishing place Paris
    Document type Book ; Online
    HBZ-ID TT050387423
    ISBN 978-2-287-77135-4 ; 978-2-287-77137-8 ; 2-287-77135-2 ; 2-287-77137-9
    DOI 10.1007/978-2-287-77137-8
    Database ZB MED Catalogue: Medicine, Health, Nutrition, Environment, Agriculture

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  7. Article ; Online: Mast cell differentiation: still open questions?

    Arock, Michel

    Blood

    2016  Volume 127, Issue 4, Page(s) 373–374

    Abstract: In this issue of Blood, Dahlin et al report on a minor circulating human mast cell (MC) progenitor cell population (lineage-negative [Lin−]/CD34hi/CD117int/hi/high-affinity immunoglobulin E receptor-positive [FcεRI+]), with an immature MC-like appearance, ...

    Abstract In this issue of Blood, Dahlin et al report on a minor circulating human mast cell (MC) progenitor cell population (lineage-negative [Lin−]/CD34hi/CD117int/hi/high-affinity immunoglobulin E receptor-positive [FcεRI+]), with an immature MC-like appearance, which is present in the peripheral blood (PB) of healthy individuals and of asthma subjects well controlled by treatment or with reduced lung function.
    MeSH term(s) Antigens, CD34/analysis ; Female ; Humans ; Male ; Mast Cells/cytology ; Proto-Oncogene Proteins c-kit/analysis ; Receptors, IgE/analysis ; Stem Cells/cytology
    Chemical Substances Antigens, CD34 ; Receptors, IgE ; Proto-Oncogene Proteins c-kit (EC 2.7.10.1)
    Language English
    Publishing date 2016-01-27
    Publishing country United States
    Document type Journal Article ; Comment
    ZDB-ID 80069-7
    ISSN 1528-0020 ; 0006-4971
    ISSN (online) 1528-0020
    ISSN 0006-4971
    DOI 10.1182/blood-2015-12-686592
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  8. Article ; Online: Tyrosine kinase inhibitors for the treatment of indolent systemic mastocytosis: Are we there yet?

    Akin, Cem / Arock, Michel / Valent, Peter

    The Journal of allergy and clinical immunology

    2022  Volume 149, Issue 6, Page(s) 1912–1918

    Abstract: Indolent systemic mastocytosis (ISM) is the most prevalent form of systemic mastocytosis. Many patients with ISM suffer from mast cell (MC) mediator-related symptoms. In a small number of patients, hematologic progression is seen in the follow-up. In ... ...

    Abstract Indolent systemic mastocytosis (ISM) is the most prevalent form of systemic mastocytosis. Many patients with ISM suffer from mast cell (MC) mediator-related symptoms. In a small number of patients, hematologic progression is seen in the follow-up. In some patients with ISM, symptoms arising from MC-derived mediators including gastrointestinal symptoms, anaphylaxis, and neuropsychiatric symptoms are kept under control with conventional mediator-targeting drugs or MC-stabilizing agents. However, in a substantial number of patients, the symptoms are refractory to such conventional therapy. For these patients, novel drugs and targeted approaches are considered. One reasonable approach may be to apply tyrosine kinase inhibitors directed against KIT and other key kinase targets expressed in neoplastic MCs in ISM. Because MCs in more than 90% of all patients with typical ISM display the KIT D816V mutant receptor, clinically effective KIT-targeting drugs have to be active against this mutant form of KIT. The 2 such most effective and well-studied agents currently available are midostaurin and avapritinib. Other KIT-targeting drugs, such as imatinib or masitinib, are less effective or even noneffective against KIT D816V and are thus recommended for use only in patients with other KIT mutant forms (noncodon 816 mutations) or with wild-type KIT. In the present article, we review the current state in the treatment of ISM with tyrosine kinase inhibitors, with special emphasis on treatment responses and potential adverse effects. In fact, all of these agents also have unique and common adverse effects, and their use to treat patients with ISM should be balanced against their toxicity and short- and long-term safety.
    MeSH term(s) Anaphylaxis/drug therapy ; Humans ; Mast Cells ; Mastocytosis/diagnosis ; Mastocytosis, Systemic/diagnosis ; Mastocytosis, Systemic/drug therapy ; Mastocytosis, Systemic/genetics ; Protein Kinase Inhibitors/therapeutic use ; Proto-Oncogene Proteins c-kit/genetics
    Chemical Substances Protein Kinase Inhibitors ; Proto-Oncogene Proteins c-kit (EC 2.7.10.1)
    Language English
    Publishing date 2022-04-26
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 121011-7
    ISSN 1097-6825 ; 1085-8725 ; 0091-6749
    ISSN (online) 1097-6825 ; 1085-8725
    ISSN 0091-6749
    DOI 10.1016/j.jaci.2022.04.020
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  9. Article ; Online: Recent Developments in the Field of Mast Cell Disorders: Classification, Prognostication, and Management.

    Valent, Peter / Arock, Michel / Akin, Cem / Metcalfe, Dean D

    The journal of allergy and clinical immunology. In practice

    2022  Volume 10, Issue 8, Page(s) 2052–2055

    MeSH term(s) Disease Progression ; Humans ; Mast Cells ; Mastocytosis/diagnosis ; Mastocytosis/therapy
    Language English
    Publishing date 2022-08-12
    Publishing country United States
    Document type Editorial ; Research Support, Non-U.S. Gov't ; Research Support, N.I.H., Intramural
    ZDB-ID 2843237-X
    ISSN 2213-2201 ; 2213-2198
    ISSN (online) 2213-2201
    ISSN 2213-2198
    DOI 10.1016/j.jaip.2022.04.041
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  10. Article ; Online: World Health Organization Classification and Diagnosis of Mastocytosis: Update 2023 and Future Perspectives.

    Valent, Peter / Sotlar, Karl / Horny, Hans-Peter / Arock, Michel / Akin, Cem

    Immunology and allergy clinics of North America

    2023  Volume 43, Issue 4, Page(s) 627–649

    Abstract: Experts of the European Competence Network on Mastocytosis (ECNM) and the American Initiative on Mast Cell Disorders have discussed and updated diagnostic criteria and the classification of mastocytosis, based on new insights in the field and data ... ...

    Abstract Experts of the European Competence Network on Mastocytosis (ECNM) and the American Initiative on Mast Cell Disorders have discussed and updated diagnostic criteria and the classification of mastocytosis, based on new insights in the field and data collected in recent years, mostly within ECNM registry projects in which studies on several thousand cases have been performed. Based on this proposal, the World Health Organization has updated its classification of mastocytosis. This article discusses the revised classification of mastocytosis in light of a rapidly moving field and the advent of new diagnostic parameters, new prognostication tools, and new therapies.
    MeSH term(s) Humans ; Mastocytosis/diagnosis ; Mastocytosis/genetics ; Mastocytosis/therapy ; World Health Organization ; Mast Cells
    Language English
    Publishing date 2023-07-18
    Publishing country United States
    Document type Journal Article ; Review ; Research Support, Non-U.S. Gov't
    ZDB-ID 92606-1
    ISSN 1557-8607 ; 0889-8561
    ISSN (online) 1557-8607
    ISSN 0889-8561
    DOI 10.1016/j.iac.2023.04.011
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