FRESH-FROZEN PLASMA PROPHYLAXIS FOR HEREDITARY ANGIOEDEMA DURING PREGNANCY - A CASE-REPORT

BACKGROUND: Hereditary angioedema (HAE) is an uncommon disorder of the complement system due to a deficiency or dysfunction of the inhibitor of the first component of complement (C1 INH). Clinically, HAE is cha racterized by episodic abdominal pain or edema of the extremities, fac e, larynx and vulva. Laryngeal edema is potentially lethal and account s for mortality rates as high as 30%. Therapy is divided into short-te rm (fresh frozen plasma) and long-term (antifibrinolytic agents, hormo nal therapy) prophylaxis, or acute treatment (epinephrine, steroids, a ntihistamines). CASE: An 18 year-old, primiparous woman presented with a history of multiple episodes of abdominal pain, swelling of abdomen and extremities. A diagnosis of hereditary angioedema was made based on the patient's and family's history and on decreased levels of C1 IN H and complement component 4. Standard prophylactic methods were contr aindicated due to potential teratogenic effects. Fresh frozen plasma g iven twice weekly over 2.5 months resulted in reduction in the number and severity of attacks and allowed successful completion of pregnancy . CONCLUSION: This is the first report of fresh frozen plasma therapy as it means of long-term prophylaxis in a pregnant woman. Fresh frozen plasma may serve as an alternate mode of long-term prophylaxis if the standard agents (antifibrinolytic, hormone) are unsuccessful or contr aindicated.