C1-estrase Inhibitor Plasma Derived

What it is and how it works

Human C1-esterase inhibitor purified from pooled plasma replaces deficient or dysfunctional endogenous C1-INH, inhibiting activated C1, kallikrein and factor XIIa. This limits bradykinin generation, the principal mediator of swelling in hereditary angioedema.

Indications

Treatment and pre-procedural prevention of acute attacks of hereditary angioedema due to C1-INH deficiency; routine prophylaxis of attacks with some products (intravenous or subcutaneous).

Dosage and administration (summary)

Dosing is individualised by the specialist according to product and indication: for acute attacks commonly a weight-based intravenous dose (e.g. 20 IU/kg) or a fixed dose of 1000 units, repeated if required; prophylactic regimens per product label.

Contraindications

Life-threatening hypersensitivity to the product or its components.

Warnings and precautions

Hypersensitivity reactions may occur; symptoms can mimic an angioedema attack. Thrombotic events have been reported, particularly with high doses or indwelling venous catheters. As a plasma-derived product it carries a residual risk of transmitting infectious agents; vaccination against hepatitis A and B should be considered for regularly treated patients.

Pregnancy

Considered the treatment of choice for HAE attacks in pregnancy when needed.

Storage and supply

Store at 2–25 °C or as specified by the manufacturer; do not freeze; keep in the outer carton to protect from light.

Source: reference summary prepared from the EU SmPC/EPAR, BNF and WHO Model Formulary. The approved product information in Iran governs.