The n e w e ng l a n d j o u r na l of m e dic i n e

TEACHING points

MANIFESTATIONS, DIAGNOSIS, AND TREATMENT OF ANAPHYLAXIS

AND SYSTEMIC MASTOCYTOSIS

• Anaphylaxis is a medical emergency in which the release of mast-cell and basophil

mediators results in life-threatening cardiovascular or respiratory compromise.
Since the features of anaphylaxis vary broadly, high clinical suspicion must be main-
tained to make sure anaphylaxis is recognized and treated promptly. Injected epi-
nephrine is the mainstay of treatment.

• Hymenoptera-sting reactions range from mild local pain and swelling to anaphylax-
is. Patients who have had a serious reaction to a hymenoptera sting should avoid
future exposure, carry injectable epinephrine, and be referred to an allergist for test-
ing and consideration of venom immunotherapy.

• Systemic mastocytosis is a disorder in which an intrinsic defect in mast cells causes

increases in mast-cell number and activity. Most patients have episodic symptoms
of activation, which may culminate in anaphylaxis. In a minority of patients there is
progression to advanced disease or the development of associated hematologic dis-
orders; in these patients, long-term complications are related to mast-cell infiltra-
tion of organs, including the liver, spleen, lymph nodes, and bone marrow, and to
the complications of the associated hematologic disorder.

• Antihistamines and the avoidance of triggers are the cornerstones of treatment for
all categories of mastocytosis. Mast-cell cytoreduction is generally reserved for
patients with advanced disease.

These teaching points are drawn from the Interactive Medical Case. For more complete information,
see Lee-Sarwar K, Vaidya A, Shi M, Akin C. A stinging sensation. N Engl J Med 2015;372:e35.