Abstract
Hereditary alpha tryptasemia was first described in 2016 and is the most common (up to 72%) cause of elevated serum basal tryptase (TPS). The clinical presentation of this condition, which is caused by copy number gains in the TPSAB1 gene encoding serum α TPS, is variable for each patient. Some patients are asymptomatic, whereas in others, especially those with increased mast cell activation, it has been associated with a higher risk of anaphylaxis. Better characterization of this entity is important to identify at-risk patients and to develop new treatment strategies. This review provided an overview of hereditary alpha tryptasemia and increased awareness of this condition by discussing the current information in the literature.