Hereditary alpha tryptasemia syndrome (HATS) is characterized by high blood tryptase levels. Symptoms of HATS are associated with multiple organ systems.These symptoms may include allergic-like symptoms such as skin itching, flushing, hives, or anaphylaxis; and gastrointestinal symptoms such as bloating, abdominal pain, diarrhea and/or constipation, heartburn, reflux, and difficulty swallowing. In addition, people with HATS may have connective tissue symptoms such as loose joints and scoliosis (often diagnosed as Ehlers-Danlos syndrome, hypermobile type) and a racing or pounding heartbeat or blood pressure swings (frequently diagnosed as postural orthostatic tachycardia syndrome (POTS). Some people with HATS also have behavioral problems.
People with hereditary alpha tryptasemia have inherited extra copies of the alpha tryptase gene (TPSAB1), which leads to increased blood levels of trypase.Tryptase is a protein made primarily by the cells that contribute to allergic responses (mast cells). When people with hereditary alpha tryptasemia have symptoms, the condition is known as “hereditary alpha tryptasemia syndrome”. People who have few or no symptoms are said to have the trait, and not the syndrome. Treatment is aimed at managing the specific symptoms and may include anti-allergy medications.
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