Functional C1-Esterase Inhibitor

Pathology category

Immunology

Code

C1EF

Referral code

RHH

Request form

Test information

Inherited or acquired defects of this protein usually result in severe angioedema, which is a painless, non-itchy swelling of sub-dermal tissues and is life-threatening if the larynx is affected. Low levels are found in 85% of cases of Hereditary Angioedema. The remaining 15% of cases are associated with a non-functioning protein, an assay for which is available. Acute attacks are treated by infusion of C1-inh concentrate or Fresh Frozen Plasma. Clinical assessment by an Immunologist is strongly recommended where this diagnosis is suspected.

Guidelines

Pre-analytical

Reference range

Tube type

Tube type info

SST, Plain or Lithium Heparin

Special requirements

Serum

Minimum volume

Assay frequency

Sent Away to a second Referral Lab. May take a further 2-3 weeks

TAT Inpatient (urgent)

TAT Inpatient

TAT GP/Outpatient

up to 4 weeks

Referral lab

Immunology Russells Hall Hospital