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 characterized by episodic abdominal pain or edema of the extremities, face, larynx and vulva. Laryngeal edema is potentially lethal and accounts for mortality rates as high as 30%. Therapy is divided into short-term (fresh frozen plasma) and long-term (antifibrinolytic agents, hormonal therapy) prophylaxis, or acute treatment (epinephrine, steroids, antihistamines).


METHODS
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 INH and complement component 4. Standard prophylactic methods were contraindicated due to potential teratogenic effects. Fresh frozen plasma given twice weekly over 2.5 months resulted in reduction in the number and severity of attacks and allowed successful completion of pregnancy.


CONCLUSIONS
This is the first report of fresh frozen plasma therapy as a 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 contraindicated.
Galan
H L
HL
Department of Obstetrics and Gynecology, Scott and White Clinic, Temple, TX 76508, USA.
Reedy
M B
MB
Starr
J
J
Knight
A B
AB
D000293:Adolescent; D000799:Angioedema / Q000150:complications / Q000235:genetics* / Q000517:prevention & control*; D001803:Blood Transfusion / Q000592:standards; D003172:Complement C1 / Q000502:physiology; D005260:Female; D005343:Fibrinolytic Agents / Q000627:therapeutic use; D006801:Humans; D010949:Plasma; D011247:Pregnancy; D011249:Pregnancy Complications, Cardiovascular / Q000517:prevention & control* / Q000628:therapy
D003172:Complement C1; D005343:Fibrinolytic Agents