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Total tryptase levels in anaphylaxis, systemic mastocytosis, and hereditary alpha-tryptasemia

Total tryptase levels in anaphylaxis, systemic mastocytosis, and hereditary alpha-tryptasemia
Clinical condition Total tryptase levels (ng/mL or mcg/L)
Normal BST ≤11.4 If patients with HaT or patients with kidney failure are excluded, then the upper limit of the normal range is 11.4 ng/mL.
Hereditary alpha-tryptasemia (HaT) BST >8 For each extra copy of TPSAB1 encoding alpha-tryptase, the BST level increases by 9 to 10 ng/mL. HaT is the most common reason for increased BST levels in those of European ancestry. Most individuals have levels between 8 and 50 ng/mL. If a subject with BST levels 6.5 to 7.9 ng/mL is suspected of having HaT and has a family member with the HaT genotype, then genotyping may be appropriate.
Kidney failure BST >6 Modest increases in BST levels have been reported, particularly in hemodialysis patients, but rarely exceed 30 ng/mL. Kidney failure is the second most common condition causing an increased BST level.
Systemic mastocytosis (SM) BST >20 Approximately 70% of SM patients have a BST >20 ng/mL, while a few have levels in the normal range. The prevalence of HaT in SM is 2- to 3-fold higher than in healthy subjects. In patients with both conditions, the BST level needs to be adjusted for the CNV*[1] but cannot be used as a diagnostic criterion when other acquired conditions that raise the BST level are present.
Anaphylaxis

Acute serum tryptase

ECNM: >1.2 × BST + 2

NIH: >1.685 × BST
A BST level of 4 ng/mL gives an equivalent threshold for each formula. The ECNM one has higher specificity and lower sensitivity at BST levels <4, while the NIH formula has higher specificity and lower sensitivity at BST levels >4. In each case, clinical judgment is needed.
Clonal myelocytic disorders (excluding SM) >10 ng/mL Examples of disorders include acute myeloid leukemia, myelocytic hypereosinophilic syndrome, myelodysplastic syndrome, and others. Levels uncommonly exceed 50 ng/mL.
The table shows causes of elevations in BST. BST should be collected either before or at least 24 hours after all signs and symptoms of a possible anaphylaxis attack have completely resolved.

BST: basal serum tryptase; CNV: copy number variant; ECNM: European Competence Network on Mastocytosis; HaT: hereditary alpha-tryptasemia; NIH: National Institutes of Health; TPSAB1: tryptase alpha/beta 1.

* NIH, subtract 9 to 10 ng/mL for each extra TPSAB1 CNV; another proposed algorithm is to divide BST by 1 + the number of CNVs.

¶ An acute serum tryptase level should be collected 30 minutes to 6 hours after onset of signs and symptoms of a possible anaphylaxis episode and optimally 30 minutes to 2 hours after onset and correlates best with the magnitude of hypotension.
Reference:
  1. Chovanec J, Tunc I, Hughes J, et al. Genetically defined individual reference ranges for tryptase limit unnecessary procedures and unmask myeloid neoplasms. Blood Adv 2023; 7:1796.
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