Case series: Hereditary angioedema patients and use of tranexamic acid treatments in prophylaxis and acute crisis – Experience in Perú

Abstract

Background: Hereditary Angioedema (HAE) is characterized by episodes of swelling at the cutaneous and submucosal levels, with acute attacks potentially leading to fatal asphyxia. Additionally, it significantly impacts the quality of life of affected individuals. There is a significant disparity in the availability of specific HAE medications across countries, even within the same region. In Peru, where these treatments are not accessible, Tranexamic Acid (TA) is used for both long-term and short-term prophylaxis (LTP/STP) as well as for acute HAE attacks.

Objective: To report the experience with TA treatment in three types of HAE patients, providing a reference for other countries where specific medications for HAE are not yet available.

Case report:

• Patient 1: A 49-year-old woman with Type 1 HAE. Symptoms began at age 12, with a definitive diagnosis at 45 years. She experiences attacks every two months and receives 2 g of intravenous (IV) TA at the onset of facial, tongue, or lip swelling. Occasionally, she requires an additional 1–2 g IV dose after four hours. She is on LTP with TA (500–750 mg) every 12 hours.
• Patient 2: A 47-year-old woman with HAE-nC1INH-FXII. Symptoms began at age 19 during her first pregnancy, with a definitive diagnosis at 41 years. She maintains LTP with TA (750 mg–1.5 g) daily. Upper airway attacks are treated immediately with IV TA (1–2 g) at the onset of symptoms.
• Patient 3: A 43-year-old woman with HAE-nC1INH-D. Genetic studies did not detect mutations in SERPING1, PLG1, ANGPT1, KNG1, or FXII. Symptoms began at age 4, with a definitive diagnosis at 36 years. During attacks, she takes 500–750 mg of oral TA every 12 hours for two to three days, showing an acceptable response and tolerance. While on TA maintenance therapy (500–750 mg every 8–12 hours), her attacks are generally milder and resolve more quickly.

Conclusions: In our HAE patients, we have used IV TA at doses of up to 4 g within a four-hour interval (2 g + 2 g) for acute attack management. When symptoms subside, we prescribe maintenance doses of 1 g every 8 hours, gradually tapering in the following days. Tranexamic acid has been beneficial for our patients with different types of HAE. Most patients require high doses of TA to gradually reduce acute attacks. It can be administered orally or intravenously and may be a useful alternative in countries lacking approved treatments for HAE. TA doses have been well tolerated, with an acceptable response in managing HAE attacks.