Fresh Frozen Plasma for the Treatment of a Chinese Patient with Hereditary Angioedema

Hereditary angioedema (HAE) caused by C1-esterase inhibitor deficiency is an autosomal-dominant disease caused by a mutation in the C1-inhibitor gene. It is a rare disease that is often worsened during pregnancy and childbirth. HAE, though uncommon but if untreated it may lead to maternal death. The case report presents the successful management of a 24 years old, G2P1, with hereditary angioedema caused by C1-esterase inhibitor deficiency. This patient was managed with a multidisciplinary approach by an obstetrician, an immunologist, an anaesthesiologist and a pediatrician. She had an uneventful antenatal period, labor was induced. She had precipitate delivery and soon after delivery had a flare up of the disease. It was successfully managed with fresh frozen plasma and close observation.

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Fresh frozen plasma for on-demand hereditary angioedema treatment in South Africa and Iran

World Allergy Organization Journal ◽

10.1016/j.waojou.2019.100049 ◽

2019 ◽

Vol 12 (9) ◽

pp. 100049 ◽

Cited By ~ 4

Author(s):

Nicole Wentzel ◽

Angelica Panieri ◽

Maryam Ayazi ◽

Sipho Duncan Ntshalintshali ◽

Zahra Pourpak ◽

...

Keyword(s):

South Africa ◽

Hereditary Angioedema ◽

Fresh Frozen Plasma ◽

On Demand ◽

Fresh Frozen ◽

Frozen Plasma

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454 Efficacy of Icatibant Versus Fresh Frozen Plasma for Attacks of Hereditary Angioedema

World Allergy Organization Journal ◽

10.1097/01.wox.0000412217.67551.bb ◽

2012 ◽

Vol 5 ◽

pp. S144-S145

Author(s):

Alicia Lara ◽

M. Esthela Hernandez

Keyword(s):

Hereditary Angioedema ◽

Fresh Frozen Plasma ◽

Fresh Frozen ◽

Frozen Plasma

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Difficult intubation due to postoperative hereditary angioedema and treatment with fresh frozen plasma

British Journal of Anaesthesia ◽

10.1093/bja/el_7108 ◽

2011 ◽

Vol 107 (eLetters) ◽

Author(s):

Sedat Saylan ◽

Ahmet Eroglu

Keyword(s):

Difficult Intubation ◽

Hereditary Angioedema ◽

Fresh Frozen Plasma ◽

Fresh Frozen ◽

Frozen Plasma

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Hereditary Angioedema: Management of Laryngeal Attacks

American Journal of Rhinology and Allergy ◽

10.2500/ajra.2011.25.3670 ◽

2011 ◽

Vol 25 (6) ◽

pp. 379-382 ◽

Cited By ~ 7

Author(s):

Sandra C. Christiansen ◽

Bruce L. Zuraw

Keyword(s):

Hereditary Angioedema ◽

Treatment Options ◽

The United States ◽

Fresh Frozen Plasma ◽

C1 Inhibitor ◽

Acute Therapy ◽

Fresh Frozen ◽

Life Threatening ◽

Frozen Plasma ◽

Future Management

Background Hereditary angioedema (HAE) patients suffering from laryngeal attacks in the United States faced severely limited treatment options until 2008. These potentially life-threatening episodes occur in over one-half of the patients affected by HAE during their lifetimes. Acute therapy had been relegated to supportive care, intubation, and consideration of fresh frozen plasma (FFP)–-the latter with the potential for actually accelerating the speed and severity of the swelling. Methods In this article we will review the recently approved and emerging HAE treatments that have evolved from the recognition that bradykinin generation is the fundamental abnormality leading to attacks of angioedema. Results Acute therapy for laryngeal attacks will be discussed including purified plasma–derived C1 inhibitor (C1INH), recombinant C1INH, an inhibitor of plasma kallikrein (ecallantide), and a B2 receptor antagonist (icatibant). Prophylactic care has also been transformed from a reliance on attenuated androgens with their attendant side effects to C1INH replacement. Conclusion The arrival of these novel therapies promises to transform the future management of HAE.

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