4.5 Review

Nontryptase Urinary and Hematologic Biomarkers of Mast Cell Expansion and Mast Cell Activation: Status 2022

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Summary: Mastocytosis is a myeloid neoplasm characterized by the accumulation of clonal mast cells in organs, leading to various symptoms and complications. The disease is categorized into different types, with KIT gene mutations associated with the condition. Early-stage cases have a better prognosis, while advanced cases have a poorer prognosis, and severe symptoms may be diagnosed as mast cell activation syndrome.

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Summary: MRGPRX2, activated by various cationic ligands, plays a role in host defense but overactivation may lead to pseudoallergy and various skin diseases. Gain and loss-of-function missense polymorphisms have been identified in MRGPRX2, and the functionality of certain ligands has been defined. The development of small-molecule HDP mimetics that activate MRGPRX2 presents a new therapeutic approach, while MRGPRX2-specific antibodies and inhibitors could be utilized for modulation of allergic and inflammatory diseases.

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Divergent PGD2 and leukotriene C4 metabolite excretion following aspirin therapy: Ten patients with systemic mastocytosis

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MRGPRX2 and Adverse Drug Reactions

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Summary: Adverse reactions to therapeutic drugs are thought to be triggered by patient-specific IgE antibodies recognizing drug molecules, but another proposed mechanism involves direct mast cell degranulation activation via MRGPRX2 receptor. Mild-to-moderate events triggered by MRGPRX2 activation may be more common than currently appreciated, even after administration of high doses for some drugs.

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Exercise-induced anaphylaxis unrelated to food ingestion and with hyperleukotrieneuria during challenge testing

Chikako Motomura et al.

Summary: This case report highlights a patient with exercise-induced anaphylaxis (EIA) diagnosed through positive exercise loading test and hyperleukotrieneuria. Cold stimulation can act as a co-effector of EIA. Measurement of urinary LTE4 levels during challenge testing is useful for diagnosing EIA and capturing the pre-anaphylaxis stage.

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Survey of Mast Cell Mediator Levels from Patients Presenting with Symptoms of Mast Cell Activation

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The Utility of Measuring Urinary Metabolites of Mast Cell Mediators in Systemic Mastocytosis and Mast Cell Activation Syndrome

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A distinct biomolecular profile identifies monoclonal mast cell disorders in patients with idiopathic anaphylaxis

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Stress triggers coronary mast cells leading to cardiac events

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Bleeding diathesis in patients with mast cell activation disease

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Increased leukotriene E4 excretion in systemic mastocytosis

Joseph H. Butterfield

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Transcellular biosynthesis of eicosanoids

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Fatal bleeding due to a heparin-like anticoagulant in a 37-year-old woman suffering from systemic mastocytosis

Christoph Sucker et al.

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Ocular mast cells and mediators

Anne-Marie A. Irani

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Prevention of Mast Cell Activation Disorder-Associated Clinical Sequelae of Excessive Prostaglandin D(2) Production

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Tetranor PGDM, an abundant urinary metabolite reflects biosynthesis of prostaglandin D2 in mice and humans

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Mast cells and eosinophils: A novel link between inflammation and angiogenesis in allergic diseases

I Puxeddu et al.

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Evidence of mast cell activation and leukotriene release after mannitol inhalation

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Serum interleukin-6 reflects disease severity and osteoporosis in mastocytosis patients

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Are adverse effects of sildenafil also caused by inhibition of diamine oxidase?

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T Matsuoka et al.

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Sputum cysteinyl leukotrienes increase 24 hours after allergen inhalation in atopic asthmatics

AJ Macfarlane et al.

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