Correct!
5. All of the above
The treatment is similar to treating anaphylaxis. He had been given epinephrine earlier at his referring hospital. He was given famotidine, montelukast, cromolyn, and a short course of corticosteroids. His clinical condition rapidly improved over 48 hours, including the severe coagulopathy.
Systemic mastocytosis may present with urticarial pigmentosa, or with symptoms related to organ infiltration by mast cells (e.g. hepatosplenomegaly), but it is most likely to present in the ICU with a syndrome caused by acute mast cell degranulation. These events are closely related to anaphylaxis, but may occur without cross-binding IgA by an allergan. Degranulation of histamine, leukotrienes, prostaglandins, cytokines cause an inflammatory cascade that results in increased vascular permeability, vasodialation, and bronchospasm. Clinically, the patients suffer cause an acute onset of flushing, pruritis, urticaria, angioedema, syncope, hypotension, abdominal pain, nausea, vomiting, and diarrhea. Degranulation events can be precipitated by a wide range of triggers that include medications (narcotics, vancomycin, radiocontrast media, etc.), surgical procedures, alcohol ingestion, hymenoptera envenomation, and even ingestion of spicy foods and emotional turmoil. Heparin and platelet-activating factor can also be degranulated, and in rare cases have caused severe coagulopathy resulting in life-threatening bleeding.
The intensivist should consider the possibility of systemic mastocystosis in any patient presenting with an acute anaphylactic-like syndrome. The history of recurrent anaphylactic episodes in a patient with urticaria pigmentosa - such as in this case - is highly suggestive of systemic mastocytosis. Urticaria pigmentosa is not likely to be readily recognized by a non-dermatologist, but Darier’s sign (the rapid development of localized wheal when the skin is stroked) can be suggestive.
Confirmation of the diagnosis of SM typically requires bone marrow biopsy. Most patients will benefit from chronic inhibition of mediator release (which can otherwise cause osteopenia) with H1 and H2 histamine blockers, and leukotriene antagonists. Triggering events should be elucidated if possible and avoided. Patients should consider the diagnosis as a most important part of their medical history, since preparation with corticosteroids and mediator-release antagonists can prevent potentially fatal degranulation events that can be triggered by medical procedures – particularly surgery. Patients with SM should be referred to an expert in this disease for ongoing care.
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