Hereditary a-tryptasemia (HaT) is a common autosomal dominant genet-ic trait with variable penetrance associated with increased serum baseline tryptase (SBT) levels. Clinical manifestations may range from an absence of symptoms to overtly severe and recurrent anaphylaxis. Symptoms have been claimed to result from excessive activation of EGF-like module -con-taining mucin-like hormone receptor-like 2 (EMR2) and ...
Background Mastocytosis encompasses a heterogeneous group of diseases characterized by tissue accumulation of clonal mast cells, which frequently includes bone involvement. Several cytokines have been shown to play a role in the pathogenesis of bone mass loss in systemic mastocytosis (SM), but their role in SM-associated osteosclerosis remains unknown. Objective To investigate the potential association between ...
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Mastocytosis is associated with a high risk of anaphylaxis, in part due to drug hypersensitivity reactions (DHR). Drugs associated with mast cell activation include nonsteroidal anti-inflammatory drugs (NSAIDs), drugs used in the perioperative setting, including general anesthetics, neuromuscular blocking agents (NMBAs) and opiates/opioids, radiocontrast media (RCM), vaccines, and antibiotics such as quinolones...
BACKGROUND: The Red Espanola de Mastocitosis (Spanish Network on Mastocytosis) score (REMAs) and the National Institutes of Health idiopathic clonal anaphylaxis score (NICAS) were developed for more efficient screening of mast cell (MC) clonality in MC activation syndromes. In a limited idiopathic anaphylaxis case series, the NICAS showed higher accuracy compared with the REMAs. OBJECTIVE: To compare the perfor...
Reported cases of anaphylaxis following COVID-19 vaccination raised concerns about the safety of these vaccines, namely in patients suffering from clonal mast cell (MC) disorders—a het-erogenous group of disorders in which patients may be prone to anaphylaxis caused by vaccination. This study aimed to assess the safety of COVID-19 vaccines in patients with clonal MC disorders. We performed an ambidirectional co...
Vaccines against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) are considered the cornerstone of the solution to the current global pandemic. The first vaccines to receive authorization for emergency use in humans were the BNT162b2 Pfizer-BioNTech and the mRNA-1273 Moderna vaccines. Both contain synthetic mRNA that codes for the SARS-CoV-2 spike (S) protein, which is encased in a lipid nanopartic...
Background: Nonsteroidal anti-inflammatory drugs (NSAIDs) are frequently avoided in mastocytosis, because of a potential increased risk for drug hypersensitivity reactions (DHRs) due to inhibition of cyclo-oxygenase (COX), subsequent depletion of prostaglandin E2 and release of leukotrienes. Objectives: Here, we aimed at determining the prevalence of mast cell (MC) mediator release symptoms triggered by NSAIDs ...
Mastocytosis encompasses a heterogeneous group of diseases characterized by the presence of clonal mast cells (MCs) in tissues and symptoms of MC activation, including anaphylaxis.1 Vaccination has been shown to cause exacerbation of MC mediator-related symptoms.2 Vaccines against coronavirus disease 2019 (COVID-19) are the solution to the current pandemic, but reports of anaphylaxis following vaccination with ...
Letter to the editor