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Hereditary angioedema type 1 (HAE-1) is the most prevalent HAE type, and it results from decreased antigenic C1 inhibitor (C1-INH) levels or function. Bradykinin-mediated angioedema in the face, trunk, extremities, genitalia, gastrointestinal tract, and upper airway are the symptoms of this rare and potentially fatal disease. PD-C1-INH concentrates, recombinant C1-INH, tranexamic acid (TXA), danazol, icatibant, ecallantide, and fresh-frozen plasma (FFP) may be used for treatment. The focus of this article is to point out D-dimer elevation during TXA use. We detected d-dimer elevation incidentally in 2 patients with hereditary angioedema (HAE) type 1 during tranexamic acid (TXA) therapy, in whom elevations were normalized a week after suspended TXA. However, the TXA treatment is expected to decrease D-dimer levels in surgery and hyperfibrinolysis. On the other hand, although using TXA in long-term prophylaxis in HAE treatment is not recommended, we observed that 2 patients had significant reduction of their attack frequency for over a year. [ABSTRACT FROM AUTHOR]

Hereditary angioedema is a rare disease with great heterogeneity of symptoms such as edema of the skin, gastro-intestinal mucosa and larynx or pharynx. Even though there are three types, the most frequent is type I, which is a result from a deficiency of the complement C1 inhibitor. The severity of its symptoms along with the low prevalence of the disease and the need for appropriate specific treatment make the diagnosis and treatment of the pathology an outstanding subject for the family physician. The present is the case of a male teenager with alpha-1 antitrypsin deficiency since he was six months old, angioedema on arms and legs since 11 years old and diagnosed with hereditary angioedema type I one year after. The definitive diagnosis of the disease enabled an appropriate treatment which consists in preventing outbreaks that may compromise the patient's life and, if they occur, administration of complement C1 inhibitor.