Fresh frozen plasma for the treatment of hereditary angioedema.
BACKGROUND
Fresh frozen plasma (FFP) has been used as a treatment option for patients with hereditary angioedema (HAE) because it contains Cl esterase inhibitor, and alternate therapies are not yet available in the United States. However, because FFP also contains other substrates, it has been hypothesized to have the potential to worsen or precipitate an acute attack of HAE.


OBJECTIVE
To research patient records and the medical literature to determine whether FFP can exacerbate symptoms or precipitate an attack of HAE.


METHODS
The following keywords were searched in PubMed and OVID: hereditary angioedema, angioedema and fresh frozen plasma, angioedema and FFP, and hereditary angioneurotic edema. English-language articles were searched from 1966 to the present. Also, after institutional review board approval, the medical records of patients with HAE at our institution who have received FFP since 1990 were reviewed to determine if there was evidence that FFP exacerbated the symptoms of HAE.


RESULTS
The English-language literature review and our patient medical record review failed to identify instances when FFP exacerbated symptoms of HAE or precipitated an attack. Several reports and our experience suggest that FFP is an effective prophylactic agent before surgery and for treatment of acute HAE attacks without evidence of exacerbation or initiation of symptoms.


CONCLUSIONS
FFP seems to be safe and effective in preventing exacerbations of HAE before surgery and for acute exacerbations of HAE without evidence of it initiating an attack or worsening a preexisting attack.
Prematta
Michael
M
Allergy, Asthma and Immunology Section, Penn State University, Hershey Medical Center, Hershey, Pennsylvania, USA. mprematta@hmc.psu.edu
Gibbs
Joshua G
JG
Pratt
Ellen L
EL
Stoughton
Tracy R
TR
Craig
Timothy J
TJ
D000328:Adult; D000368:Aged; D000799:Angioedema / Q000235:genetics / Q000503:physiopathology / Q000628:therapy*; D003174:Complement C1 Inactivator Proteins / Q000627:therapeutic use; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010949:Plasma; D010951:Plasma Exchange; D016896:Treatment Outcome
D003174:Complement C1 Inactivator Proteins