From the Guidelines
Medications do not typically cause mastocytosis, which is a rare disorder characterized by the abnormal accumulation of mast cells in one or more organ systems. Mastocytosis is generally considered a genetic or acquired condition rather than a medication-induced disorder. The condition is often associated with mutations in the KIT gene, particularly the D816V mutation, which leads to abnormal mast cell growth and accumulation. While medications don't cause mastocytosis, certain drugs can trigger mast cell degranulation in patients who already have mastocytosis, leading to symptom flares. These include:
- NSAIDs like aspirin and ibuprofen
- Opioid analgesics
- Alcohol
- Certain antibiotics (particularly vancomycin and fluoroquinolones)
- Radiocontrast media
- Some anesthetic agents, such as atracurium and mivacurium, which should be avoided in favor of safer alternatives like propofol, sevoflurane, or isoflurane 1. Patients with mastocytosis should maintain a list of medications that have previously caused reactions and discuss any new medications with their healthcare provider before taking them. Understanding these medication triggers is important for symptom management, though they are not causative factors for the underlying disease. In the event of anaphylaxis or other mast cell activation event, a full allergic workup should be initiated, including measurement of baseline serum tryptase level after full recovery, and identification of IgE-mediated hypersensitivity to drugs or latex requires detection of specific IgE and skin testing (skin prick and intradermal tests) 1. Pre-anesthetic treatment with anxiolytic agents, antihistamines, and possibly corticosteroids can help reduce the frequency and severity of mast cell activation events 1. It is essential to carefully review prior anesthetic records and identify/avoid known triggers of mast cell activation to prevent mast cell activation during and in the immediate aftermath of the surgical procedure 1.
From the Research
Medications that May Cause or Exacerbate Mastocytosis
- Nonsteroidal anti-inflammatory drugs (NSAIDs) are the most common elicitors of drug-induced anaphylaxis (DIA) in patients with mastocytosis, accounting for 56% of cases 2
- Perioperative agents and antibiotics can also trigger anaphylactic reactions in patients with mastocytosis, with frequencies of 23% and 13%, respectively 2
- Certain tyrosine kinase inhibitors, such as imatinib, may be less effective or even ineffective against the KIT D816V mutant receptor, which is present in more than 90% of patients with typical indolent systemic mastocytosis (ISM) 3, 4, 5
- Other medications that may be used to treat mastocytosis, such as midostaurin and avapritinib, can have unique and common adverse effects, and their use should be balanced against their toxicity and short- and long-term safety 3
Treatment Strategies for Mastocytosis
- The mainstay of treatment for most categories of mastocytosis are H1 and H2 antihistamines, with the addition of corticosteroids for more severe symptoms 6
- Symptomatic treatment may also comprise anti-leukotrienes, proton pump inhibitors, and topical corticosteroids 5
- Cytoreductive therapy, including interferon alpha 2b and tyrosine kinase inhibitors, may be necessary for indolent mastocytosis with refractory symptoms, aggressive mastocytosis with organ dysfunction, and mast cell leukemia 4, 5