Anaphylaxis and Mastocytosis

Question 1

potentially life-threatening systemic allergic reaction involving one or more organ systems that typically occurs within seconds to minutes of exposure to the anaphylactic trigger, most often a drug, food, or hymenoptera sting

Answer 1

Anaphylaxis

Question 2

among the most common presentations of anaphylaxis (>90% of cases)

Answer 2

Cutaneous manifestations

Question 3

more practical and useful biomarker

Answer 3

Serum tryptase

Question 4

Serum tryptase peaks ____ min and duration _____ hours

Answer 4

peaks at 60–90 min

Duration: 5 hours

Question 5

Treatment of choice for anaphylaxis

Answer 5

Intramuscular 0.3–0.5 mL of 1:1000 (1 mg/mL) epinephrine

Question 6

Complication of IM epinephrine administration

Answer 6

“empty heart syndrome”, insufficient venous return to the heart from sudden onset hypotension secondary to intravascular volume depletion

Question 7

elicits a temporary state of tolerance to the drug in sensitized, clinically reactive patients

Answer 7

Desensitization

Question 8

Typical premedication regimen for radiocontrast

Answer 8

Prednisone 0.5 mg/kg at 13, 6, and 1 h prior to contrast.

Diphenhydramine 25 mg is also given 1 h prior to contrast.

Question 9

defined by accumulation of clonally expanded mast cells in tissues such as skin, bone marrow, liver, spleen, and gut

Answer 9

Mastocytosis

Question 10

Majority form of mastocytosis in adults

Answer 10

Indolent systemic mastocytosis

Question 11

C-findings for diagnosis of SSM and ASM.

Answer 11

C

1. Cytopenia(s): ANC<1,000/μL or Hb<10 g/dL or PLT<100,000/μL
2. Hepatomegaly with ascites and impaired liver function
3. Palpable splenomegaly with associated hypersplenism
4. Malabsorption with hypoalbuminemia and weight loss
5. Skeletal lesions: large area(s) of osteolyses with pathologic fractures (presence of osteoporosis alone without osteolytic lesions does not s