AHA Coding Clinic® for ICD-10-CM and ICD-10-PCS - 2021 Issue 4; ICD-10-CM New/Revised Codes

Hereditary Alpha Tryptasemia

Subcategory D89.4, Mast cell activation syndrome and related disorders, has been expanded with the creation of code D89.44, Hereditary alpha tryptasemia. Hereditary alpha tryptasemia (HαT) is a genetic trait that is characterized by an elevated basal serum tryptase, a mast cell mediator, and increased copies of the gene TPSAB1. The increase of tryptase and copies of TPSAB1 are associated with multiple complaints, such as skin flushing, pruritus, dysautonomia (dysfunction of the autonomic nervous system), chronic pain, and connective tissue abnormalities. Those with hereditary alpha tryptasemia have an increased risk of severe allergic reactions to stinging...

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