Hereditary Angioedema (HAE)

CPT: 86160(x2). If reflex testing is performed, concomitant CPT codes/charges will apply.
Updated on 11/16/2018
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Test Details

Synonyms

  • Angioedema Profile
  • C1 Esterase Inhibitor Deficiency Profile

Test Includes

Complement C4, serum; complement C1 esterase inhibitor, serum; and automatic reflex to complement C1 esterase inhibitor, functional (at additional charge) if the C1 esterase inhibitor result is ≥21 mg/dL.

Use

Aid clinicians in obtaining an appropriate diagnosis of hereditary angioedema (HAE). The profile begins with complement C4 and complement C1 esterase inhibitor with reflexing, if required, to complement C1 esterase inhibitor, functional to distinguish HAE type I from type II. If the results are not consistent with HAE consider other types of angioedema and/or retesting during an acute attack.

Limitations

Rheumatoid factors (>200 IU/mL) significantly increase the apparent C4 concentration. This profile is not intended for the diagnoses of estrogen-dependent, estrogen-associated, drug-induced, or idiopathic angioedema. Also note that patients taking anabolic steroids may have falsely elevated C4 values thus limiting the effectiveness of the profile's logic. Consider retesting the patient after discontinuation of anabolic steroids.

Methodology

Immunologic

Specimen Requirements

Specimen

Serum, frozen and room temperature

Volume

Three aliquots: one tube 0.5 mL frozen, two tubes 1 mL each at room temperature

Two aliquots: 0.5 mL frozen, 2 mL at room temperature

Three aliquots: one tube 0.5 mL frozen, two tubes 1 mL each at room temperature

Minimum Volume

Three aliquots: one tube 0.1 mL frozen, two tubes 0.5 mL each at room temperature

Two aliquots: 0.1 mL frozen, 1 mL at room temperature

Three aliquots: one tube 0.1 mL frozen, two tubes 0.5 mL each at room temperature

Container

Gel-barrier tube (preferred) or red-top tube. Note: Since specimen must be drawn on ice, plain red-top tubes will not clot as well as the use of clot-activated gel tubes.

Collection

Collect sample on ice. Separate serum from cells in a refrigerated centrifuge. If a refrigerated centrifuge is not available, chill centrifuge tube carriers before centrifuging. Transfer aliquots of serum to each of three plastic transport tubes. Freeze the required 0.5 mL frozen aliquot immediately at -20°C.

Collect sample on ice. Separate serum from cells in a refrigerated centrifuge. If a refrigerated centrifuge is not available, chill centrifuge tube carriers before centrifuging. Transfer aliquots of serum to each of two plastic transport tubes. Freeze the required 0.5 mL frozen aliquot immediately at -20°C.

Collect sample on ice. Separate serum from cells in a refrigerated centrifuge. If a refrigerated centrifuge is not available, chill centrifuge tube carriers before centrifuging. Transfer aliquots of serum to each of three plastic transport tubes. Freeze the required 0.5 mL frozen aliquot immediately at -20°C.

Storage Instructions

Frozen sample should not be stored at room temperature (15°C to 30°C) for longer than six hours. Required room temperature samples are stable for three days, frozen samples for 14 days.

Causes for Rejection

Plasma specimen; specimen not drawn on ice; stored specimen not frozen; gross bacterial contamination; excess lipemia; hemolysis; separate specimens not received (room temperature and frozen)

Clinical Information

References

Bowen T, Cicardi M, Bork K, et al. Hereditary angioedema: A current state-of-the-art review, VII: Canadian Hungarian 2007 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema. Ann Allergy Asthma Immunol. 2007; 100(1Suppl 2):S30-S34. 18220150
Bowen T, Cicardi M, Farkas H, et al. Canadian 2003 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema. J Allergy Clin Immunol. 2004; 114(3):629-637. 15356569
National Organization for Rare Disorders. The Physician's Guide to Hereditary Angioedema. Danbury, Conn: NORD; 2007.
Weiler CR, van Dellen RG. Genetic test indications and interpretations in patients with hereditary angioedema. Mayo Clin Proc. 2006 Jul; 81(7):958-972. 16835976

LOINC® Map

Order Code Order Code Name Order Loinc Result Code Result Code Name UofM Result LOINC
123020 Hereditary Angioedema (HAE) 001833 Complement C4, Serum mg/dL 4498-2
123020 Hereditary Angioedema (HAE) 004649 C1 Esterase Inhibitor, Serum mg/dL 4477-6
Reflex Table for C1 Esterase Inhibitor, Serum
Order Code Order Name Result Code Result Name UofM Result LOINC
Reflex 1 120219 C1 Esterase Inhibitor, Func 120222 C1 Est.Inhib.Funct. %mean normal 10634-4
Reflex 2 000000 120223 000000 N/A
Reflex Table for C1 Esterase Inhibitor, Serum
Order Code Order Name Result Code Result Name UofM Result LOINC
Reflex 1 120219 C1 Esterase Inhibitor, Func 120222 C1 Est.Inhib.Funct. %mean normal 10634-4
Reflex 2 000000 120224 000000 N/A
Reflex Table for C1 Esterase Inhibitor, Serum
Order Code Order Name Result Code Result Name UofM Result LOINC
Reflex 1 120219 C1 Esterase Inhibitor, Func 120222 C1 Est.Inhib.Funct. %mean normal 10634-4
Reflex 2 000000 120225 000000 N/A
Reflex Table for C1 Esterase Inhibitor, Serum
Order Code Order Name Result Code Result Name UofM Result LOINC
Reflex 1 120226 HAE Interpretation: 120226 HAE Interpretation: N/A

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The LOINC® codes are copyright © 1994-2018, Regenstrief Institute, Inc. and the Logical Observation Identifiers Names and Codes (LOINC) Committee. Permission is granted in perpetuity, without payment of license fees or royalties, to use, copy, or distribute the LOINC® codes for any commercial or non-commercial purpose, subject to the terms under the license agreement found at https://loinc.org/license/. Additional information regarding LOINC® codes can be found at LOINC.org, including the LOINC Manual, which can be downloaded at LOINC.org/downloads/files/LOINCManual.pdf