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Mastocytosis with bee sting anaphylaxis

Question:

1/19/2017
A 76 year-old Caucasian male with confirmed Mastocytosis (Serum Tryptase Levels > 150 for last 7 years and + C kit Mutation) presents with passing out s/p bee sting in his foot within 15 minutes. Was taken to hospital, resuscitated and discharged without admission. PMH is notable for chronic sinus issues, penicillin allergy and no asthma. Mastocytosis is indolent and clinically stable with only pruritis (patient is taking 10 mg Claritin bid). Patient has Epinephrine. What is the best option and recommendation for this patient?

Answer:

Patients with mastocytosis as well as mast cell activation disorders are at increased risk of anaphylaxis following insect stings. Although they are also at greater risk of reaction to allergen immunotherapy, this would be the desirable therapy as the risk of a reaction to supervised immunotherapy is preferable to unsupervised field stings. You should document a discussion emphasizing the increased risk for anaphylaxis in general and the need to keep an epinephrine autoinjector readily available. We all feel apprehension about the use of epinephrine in older individuals, but any increase of risk is less than the risk of untreated anaphylaxis and hypotension. You do not mention, but I would recommend that beta blocker therapy be avoided if possible and I would also prefer that angiotensin converting enzyme inhibitors be avoided. Angiotensin receptor blockers may also interfere with the physiologic response to hypotension so I would ask that these agents not be used unless specific need is documented.

I have copied some excerpts from the Practice Parameter on Insect Allergy below. I also provided a reference from JACI: In Practice that you may find of interest. The summary is that tryptase elevation or mastocytosis is a marker for risk of more severe insect allergy and identifies need for treatment and longer term venom or imported fire ant whole body extract immunotherapy.

Lieberman P, Schwartz LB. Anaphylactic reaction to white-faced hornet sting and elevated baseline (asymptomatic) serum tryptase. J Allergy Clin Immunol: In Practice 2013;1:315

Golden, D. B., et al. "Stinging insect hypersensitivity: A practice parameter update 2016."Annals of Allergy, Asthma & Immunology: official publication of the American College of Allergy, Asthma, & Immunology 118.1 (2017): 28.

"In contrast, patients with a history of anaphylaxis to a sting have an average of almost 50% 489 frequency of systemic reaction to a sting. Patients with large local reactions have less than 10% chance of a systemic reaction (and less than 5% chance of anaphylaxis). However, no test 491 predicts the severity of a sting reaction (other than basal serum tryptase). "

"Compared to other causes of anaphylaxis such as foods or medications, the prevalence of mast cell disorders is higher in patients who have had anaphylaxis to an insect sting. Therefore, measurement of basal serum tryptase should be considered in all patients who are candidates for VIT. Elevated basal serum tryptase is closely correlated with the risk of severe anaphylaxis to stings, and is most frequently found in patients with reactions including hypotensive shock. The frequency of abnormal basal tryptase is much lower in patients with less severe systemic reactions to stings, and the clinical significance in these patients is less clear. There is a cost and burden associated with abnormal results of basal tryptase (eg: bone marrow biopsy, consultation with other specialists, anxiety associated with an abnormal test result). However an abnormal result is associated with severe anaphylaxis to stings, increased risk of systemic reactions during VIT (to a sting or venom injection), and greater risk of sting anaphylaxis after stopping VIT. With these considerations in mind, measurement of basal serum tryptase is highly recommended in patients who had hypotensive reactions to a sting, and should be considered in other patients with systemic reactions to stings. Additionally, elevated basal tryptase may indicate the presence of an occult mast cell disorder and may be present in sting-allergic patients with negative venom allergy tests as well."

"Encourage continuation of VIT for an extended time, or indefinitely, in patients with high-risk factors, such as very severe reaction before VIT (syncope, hypotension, severe respiratory distress), systemic reaction during VIT, honeybee allergy, and increased basal serum tryptase levels. (Strong Recommendation; C evidence)"

I hope this information is of help to you and your practice.

All my best.
Dennis K. Ledford, MD, FAAAAI