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Test ID HGBCE Hemoglobin Variant, A2 and F Quantitation, Blood

Useful For

Monitoring patients with sickling disorders who have received hydroxyurea or transfusion therapy

 

This test is not intended for diagnostic purposes.

 

This test is not useful for screening purposes.

Method Name

Capillary Electrophoresis

Reporting Name

Hb Variant, A2 and F Quantitation,B

Specimen Type

Whole Blood EDTA


Ordering Guidance


This test is intended for monitoring purposes, such as the increase in hemoglobin F after therapy or the levels of hemoglobin variants after transfusion.

 

If the patient has never been appropriately studied, hemoglobin electrophoresis is necessary; see HBEL1 / Hemoglobin Electrophoresis Evaluation, Blood.

 

Multiple hematology evaluations are available. For information on testing that is performed with each evaluation, see Benign Hematology Evaluation Comparison.



Specimen Required


Container/Tube:

Preferred: Lavender top (EDTA)

Acceptable: Yellow top (ACD) or green top (heparin)

Specimen Volume: 4 mL

Collection Instructions:

1. Submit fresh specimen.

2. Send whole blood specimen in original tube. Do not aliquot.


Specimen Minimum Volume

1 mL

Specimen Stability Information

Specimen Type Temperature Time Special Container
Whole Blood EDTA Refrigerated 10 days

Clinical Information

The treatment of red blood cell sickling disorders may involve many therapeutic modalities. Two of the most important and beneficial are treatment with hydroxyurea and chronic transfusion therapy. Hydroxyurea causes elevation of fetal hemoglobin (HbF) levels, and transfusion serves to lower the percentage of hemoglobin S (HbS). Both of these therapeutic modalities act to lessen the number and severity of sickling crises. Thus, periodic monitoring of HbF and HbS levels are needed to guide further therapy.

Reference Values

HEMOGLOBIN A

0-30 days: 5.9-77.2%

1-2 months: 7.9-92.4%

3-5 months: 54.7-97.1%

6-8 months: 80.0-98.0%

9-12 months: 86.2-98.0%

13-17 months: 88.8-98.0%

18-23 months: 90.4-98.0%

≥24 months: 95.8-98.0%

 

HEMOGLOBIN A2

0-30 days: 0.0-2.1%

1-2 months: 0.0-2.6%

3-5 months: 1.3-3.1%

≥6 months: 2.0-3.3%

 

HEMOGLOBIN F

0-30 days: 22.8-92.0%

1-2 months: 7.6-89.8%

3-5 months: 1.6-42.2%

6-8 months: 0.0-16.7%

9-12 months: 0.0-10.5%

13-17 months: 0.0-7.9%

18-23 months: 0.0-6.3%

≥24 months: 0.0-0.9%

 

VARIANT 1

0.0

 

VARIANT 2

0.0

 

VARIANT 3

0.0

Interpretation

Clinically, optimal levels of hemoglobin (Hb) S and fetal hemoglobin (HbF) are patient specific and depend on a number of factors including response to therapy. This test will be performed by capillary electrophoresis and any detected variant present will be reported as their zone only, including HbS. No confirmatory functional study, such as sickle solubility, will be performed as this test is designed for quantitative monitoring of previously confirmed hemoglobin fractions.

 

Information reported: Percentages of HbA, HbA2, HbF and any detected hemoglobin variant present. Variants will be reported as zones and are not specific, even if present in Z5 (Zone S). If the identity of the variant has not been previously confirmed, diagnostic hemoglobin electrophoresis testing is necessary; see HBEL1 / Hemoglobin Electrophoresis Evaluation, Blood.

Clinical Reference

1. Riou J, Szuberski J, Godart C, et al: Precision of CAPILLARYS 2 for the detection of hemoglobin variants based on their migration positions. Am J Clin Pathol. 2018 Jan 29;149(2):172-180

2. National Heart, Lung, and Body Institute Expert Panel: Evidence-Based Management of Sickle Cell Disease: Expert Panel Report, 2014. NIH Publication No. 02-2117 US Department of Health and Human Services: National Institutes of Health; 2014:1-142

3. Rosse WF, Telen M, Ware R: Transfusion Support for Patients with Sickle Cell Disease. American Association of Blood Banks; 1998

4. Ferster A, Tahriri P, Vermylen C, et al: Five years of experience with hydroxyurea in children and young adults with sickle cell disease. Blood. 2001;97:3268-3632

5. Charache S, Terrin ML, Moore RD, et al: Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia. N Engl J Med. 1995 May;332(20):1317-1322

6. Keren DF, Shalhoub R, Gulbranson R, Hedstrom D: Expression of hemoglobin variant migration by capillary electrophoresis relative to hemoglobin A2 improves precision. Am J Clin Pathol. 2012 Apr;137(4):660-664

Day(s) Performed

Monday through Friday

Report Available

Same day/1 to 2 days

Test Classification

This test has been modified from the manufacturer's instructions. Its performance characteristics were determined by Mayo Clinic in a manner consistent with CLIA requirements. This test has not been cleared or approved by the US Food and Drug Administration.

CPT Code Information

83020

LOINC Code Information

Test ID Test Order Name Order LOINC Value
HGBCE Hb Variant, A2 and F Quantitation,B 43113-0

 

Result ID Test Result Name Result LOINC Value
41927 Hb A 20572-4
41928 Hb F 4576-5
41929 Hb A2 4551-8
41930 Variant 1 24469-9
41931 Variant 2 24469-9
41932 Variant 3 24469-9
41933 HGBCE Interpretation 78748-1

Forms

1. Metabolic Hematology Patient Information (T810)

2. If not ordering electronically, complete, print, and send a Benign Hematology Test Request Form (T755) with the specimen.