How is mast cell release managed?

From the Guidelines

Mast cell release is best managed through a multidisciplinary approach, including the use of anxiolytic agents, antihistamines, and corticosteroids to prevent mast cell activation, as well as having a plan in place for emergency situations such as anaphylaxis, which may involve the administration of epinephrine. The management of mast cell release involves several key components, including:

• Preventing mast cell activation during surgical procedures by avoiding known triggers and using certain perioperative drugs that are considered safer in patients with systemic mastocytosis, such as propofol, sevoflurane, or isoflurane, as well as analgesics like fentanyl or remifentanil 1.
• Using medications such as benzodiazepines, antihistamines (H1 and H2 blockers), and corticosteroids to reduce the frequency and/or severity of mast cell activation symptoms, as these have been shown to be probably helpful in this context 1.
• Having an emergency action plan in place, which includes the administration of epinephrine for severe reactions, as well as fluid resuscitation and discontinuation of the suspected drug or anesthetic agent 1.
• Conducting a full allergic workup in the event of anaphylaxis or other mast cell activation event, which should include skin tests or detection of specific IgE antibodies and measurement of serum tryptase level within 30 to 120 minutes of onset of symptoms and also after full recovery 1. It is also important to note that the risk of anaphylaxis in the perioperative period is estimated to be higher in patients with systemic mastocytosis relative to the general population, and therefore, caution should be exercised when using certain perioperative drugs, such as opioids, which can trigger mast cell activation 1.

From the FDA Drug Label

In vitro and in vivo animal studies have shown that cromolyn sodium inhibits the release of mediators from sensitized mast cells. Cromolyn sodium acts by inhibiting the release of histamine and leukotrienes (SRS-A) from the mast cell.

Mast cell release is managed by inhibiting the release of mediators from sensitized mast cells using cromolyn sodium 2. The key benefits of using cromolyn sodium include:

• Inhibition of histamine and leukotrienes (SRS-A) release from the mast cell
• Improvement in symptoms such as diarrhea, flushing, headaches, vomiting, urticaria, abdominal pain, nausea, and itching in patients with mastocytosis 2

From the Research

Mast Cell Release Management

Mast cell release can be managed through various approaches, including:

• Blockade of mediator receptors, such as H1 and H2 antihistamines, and leukotriene receptor blockade 3
• Inhibition of mediator synthesis, including aspirin and zileuton 3
• Inhibition of mediator release, such as sodium cromolyn 3
• Anti-IgE therapy 3
• A combination of these approaches 3

Treatment Options

Treatment options for mast cell activation syndrome (MCAS) and systemic mastocytosis (SM) include:

• Epinephrine for acute episodes of mast cell activation 3
• Corticosteroids for prolonged episodes 3
• Mast cell mediator blockers, mast cell stabilisers, and anti-inflammatory agents 4
• Topical antihistamines and mast cell stabilisers for treating seasonal and perennial allergic conjunctivitis 5

Diagnostic Approaches

Diagnostic approaches for suspected mast cell activation syndrome (MCAS) include:

• A proposed diagnostic algorithm to suspect and document or exclude MCAS 6
• Consideration of the two principal diagnoses that may underlie MCAS, namely, severe allergy and systemic mastocytosis accompanied by severe mast cell activation 6

Pharmacotherapy

Pharmacotherapy for mast cell disorders (MCDs) is mainly directed against the effects of mast cells and their mediators, and may include:

• Targeted therapies aiming at blocking mutant KIT variants and/or downstream signaling pathways 7
• A stepwise, individual-based approach in pharmacotherapy options, with the core component being to control symptoms caused by mast cell mediator release 7