Daily Life Activity Creates Triggers in Mast Cell Disorders

Mast cell disorders (MCDs) involve unpredictable, disabling symptoms that interfere with daily life activities, according to a recently published overview of this little-understood condition.

A recent Perspective in Annals of Allergy, Asthma, and Immunology described the many challenges faced by patients with MCD and said that optimized care can be guided by their viewpoints.

Mast cells (MCs) are immune system cells that live in the bone marrow and internal and external tissues, including the gastrointestinal tract, airway lining, and the skin. MC activation (MCA) may be induced spontaneously or by triggers, releasing mediators such as tryptase, histamine, leukotrienes, and prostaglandins. Multiple organs can be affected and anaphylaxis is possible.

Symptoms in clonal and nonclonal MCDs include mastocytosis, MCA syndrome (MCAS), and hereditary alpha tryptasemia (HαT), which is also a modifier of MCD.

Signs and symptoms may result from proliferation and accumulation of abnormal MCs in clonal MCDs and range from acute, episodic, and recurrent to chronic and frequent.

Some patients with MCAS, HαT, or both MCAS and HαT may also have dysautonomia symptoms and connective tissue abnormalities.

To meet the proposed diagnostic criteria for MCAS, symptoms must also be severe, be present concurrently in 2 or more organ systems, and respond to antimediator or MC-stabilizing therapy, as well as antihistamines.

The list of potential triggers is long, which means that in their efforts to avoid them, patients with MCA can experience psychological stress and social isolation, affecting their quality of life. Triggers may be set off by ingestion, touch, inhalation, or even just the experience of them (such as stress or temperature changes).

Triggers include:

• Venoms, which can lead to fatal anaphylaxis
• Medications, contrast media, and vaccines
• Excipients in medications
• Alcohol, including in topical, oral, inhaled, and injectable medications and in hand sanitizers and cleaners
• Stress, including emotional, environmental, psychosocial, and physical, such as friction from clothing
• Major and minor surgery and invasive or radiologic procedures

Other triggers are foods, beverages, and alcohol, and trying to avoid those may increase the risk of social isolation.

For instance, a survey conducted by a patient organization of approximately 1600 respondents with an MCAS diagnosis found that 41% reported being restricted to 20 foods or less, while the majority (71%) indicated food limitations overall.

Patients find treatment options—which include drugs aimed at preventing MCA as well as controlling the release of mediators—to have varying degrees of effectiveness. These include H1 and H2 blockers, cromolyn sodium, leukotriene inhibitors, aspirin, and corticosteroids.

In addition, omalizumab, which reduces immunoglobulin E, was reported as effective by 61% of the 17% of patients who were currently taking or had taken it.

Because of the risk of anaphylaxis, having 2 doses of self-injectable epinephrine available at all times is required.

“Managing multiple medications is an additional stressor. Successful medication management is highly dependent on access to and collaboration with a physician well informed on MCDs who can make appropriate medication adjustments,” the author wrote.

Reference

Jennings SV, Slee VM, Finnerty CC, Hempstead JB, Bowman AS. Symptoms of mast cell activation: the patient perspective. Ann Allergy Asthma Immunol. 2021;127(4):407-409. doi:10.1016/j.anai.2021.07.004