4.6 Review

A Puzzling Mast Cell Trilogy: Anaphylaxis, MCAS, and Mastocytosis

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Summary: This study compared the performance of REMAs and NICAS in the diagnosis of MC clonality. The results showed that REMAs had higher accuracy and rates of correctly classified patients compared to NICAS, especially in male patients, those with systemic mastocytosis, and those with anaphylaxis featuring urticaria, cardiovascular symptoms, and/or presyncope. It is recommended to combine REMAs and blood detection of KITD816V to improve classification efficiency.

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Summary: This study found that the ratios of acute/baseline levels of urinary mediator metabolites in MCAS patients were around 1.3 when there was a 20% plus 2 ng/mL increase in serum tryptase. This suggests that an acute increase of 1.3 or higher in these mediators can be useful for confirming a diagnosis of MCAS.

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Summary: Mastocytosis is a heterogeneous disease characterized by uncontrolled proliferation and increased density of mast cells. In this study, it was found that serum concentrations of Thymic stromal lymphopoietin (TSLP) were lower in patients with mastocytosis compared to healthy donors. TSLP was found to be a substrate for human mast cell tryptase, indicating a novel connection between these mediators in mastocytosis.

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Summary: This study found that serum levels of TIM-3 and galectin-9 are increased in patients with advanced systemic mastocytosis. Additionally, TIM-3 and galectin-9 are expressed in bone marrow infiltrates in mastocytosis. These findings suggest that TIM-3 and galectin-9 could be explored as diagnostic markers and potential therapeutic targets in systemic mastocytosis, particularly in advanced forms.

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Diagnostic Evaluation of Hypersensitivity Reactions to Antibiotics in a Large Cohort of Mastocytosis Patients

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Summary: This study investigates the prevalence and clinical features of hypersensitivity reactions to antibiotics in mastocytosis patients. The results suggest a similar prevalence of antibiotic hypersensitivity in these patients compared to the general population, and antibiotics appear to rarely cause anaphylaxis in this population. Therefore, mastocytosis patients without a history of antibiotic hypersensitivity may be safely treated with these drugs.

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Summary: Indolent systemic mastocytosis (ISM) is the most common form of systemic mastocytosis, and many patients experience symptoms related to mast cell mediators. Conventional medication may effectively control symptoms in some patients, but there is a subset of patients who do not respond to these treatments. Tyrosine kinase inhibitors directed against specific gene mutations may be an alternative option for these patients.

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Peter Valent et al.

Summary: Mast cell activation (MCA) is a common physiological pathological phenomenon that occurs in many diseases. In some patients, there is no obvious underlying disease or trigger for MCA. When symptoms are severe, systemic, and recurrent, and accompanied by an increase in serum tryptase level or other mediators of mast cells, it may be diagnosed as mast cell activation syndrome (MCAS).

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

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Summary: Quantification of urinary metabolites can improve diagnosis and management of patients with systemic mastocytosis and mast cell activation syndrome. It provides a more accurate and targeted approach for diagnosis and treatment.

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Mastocytosis presenting with mast cell-mediator release-associated symptoms elicited by cyclo oxygenase inhibitors: prevalence, clinical, and laboratory features

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Georg Greiner et al.

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Jonathan J. Lyons et al.

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

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John M. Kelso

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