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Current Allergy and Asthma Reports

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01-05-2021 | Anaphylaxis | Anaphylaxis and Drug Allergy (BT Kelly and M Castells, Section Editors)

Hereditary Alpha-Tryptasemia: a Commonly Inherited Modifier of Anaphylaxis

Authors: Richard Wu, Jonathan J. Lyons

Published in: Current Allergy and Asthma Reports | Issue 5/2021

Abstract

Purpose of Review

Hereditary alpha-tryptasemia (HαT) is an autosomal dominant genetic trait and a common cause of elevated basal serum tryptase in Western populations. It is a risk factor for severe anaphylaxis among individuals with venom allergy and an established modifier of anaphylaxis and mast cell mediator–associated symptoms among patients with systemic mastocytosis. Understanding the physiology of tryptases and how this may relate to the clinical features associated with HαT is the first step in identifying optimal medical management and targets for novel therapeutics.

Recent Findings

HαT prevalence is increased in both clonal and non-clonal mast cell–associated disorders where it augments symptoms of immediate hypersensitivity, including anaphylaxis. The unique properties of naturally occurring α/β-tryptase heterotetramers may explain certain elements of phenotypes associated with HαT, though additional mechanisms are being evaluated.

Summary

This review provides an overview of the clinical and translational studies that have identified HαT as a modifier of mast cell–associated disorders and anaphylaxis and discusses mechanisms that may potentially explain some of these clinical findings.

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Title: Hereditary Alpha-Tryptasemia: a Commonly Inherited Modifier of Anaphylaxis
Authors: Richard Wu
Jonathan J. Lyons
Publication date: 01-05-2021
Publisher: Springer US
Keywords: Anaphylaxis
Mastocytosis
Systemic Mastocytosis
Anaphylaxis
Published in: Current Allergy and Asthma Reports / Issue 5/2021
Print ISSN: 1529-7322
Electronic ISSN: 1534-6315
DOI: https://doi.org/10.1007/s11882-021-01010-1

At a glance: The STEP trials

Springer Medicine

Hereditary Alpha-Tryptasemia: a Commonly Inherited Modifier of Anaphylaxis

Abstract

Purpose of Review

Recent Findings

Summary

At a glance: The STEP trials

Springer Medicine

Abstract

Purpose of Review

Recent Findings

Summary

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