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Mature and total tryptase levels in anaphylaxis, systemic mastocytosis, and alpha-tryptasemia

Mature and total tryptase levels in anaphylaxis, systemic mastocytosis, and alpha-tryptasemia
Clinical condition Tryptase levels (ng/mL) Total/mature tryptase ratio
Total Mature
Normal 1 to 11.4* <1 Not applicable
Systemic anaphylaxis (acute) >1.2 baseline + 2 >1Δ <10
Systemic mastocytosis (nonacute) >20Δ <1 >20
Hereditary alpha-tryptasemia  ≥8  <1  Not applicable
* Reference ranges may vary slightly among different laboratories.
¶ Level related to clinical severity (hypotension), timing of sample collection in relation to onset of signs and symptoms, and nature of the anaphylactic stimulus.
Δ Reflects primarily the total body burden of mast cells when subject is in a nonanaphylactic state. A baseline total tryptase level >11.4 ng/mL should raise the possibility of an underlying clonal mast cell disorder (eg, systemic mastocytosis), particularly in a subject with a history of systemic anaphylaxis or a primary mast cell activation disorder.
Reflects increased TPSAB1 gene copy number when that gene encodes alpha-tryptase, leading to increased formation of alpha/beta-tryptase heterotetramers, which in turn may account for severe episodes of spontaneous or allergen-triggered systemic anaphylaxis.
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