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HelpTest 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.
Special Instructions
Method Name
Capillary Electrophoresis
Reporting Name
Hb Variant, A2 and F Quantitation,BSpecimen Type
Whole Blood EDTAOrdering 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;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;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;137(4):660-664
Day(s) Performed
Monday through Friday
Report Available
Same day/1 to 2 daysTest 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 | 32682-7 |
| 41929 | Hb A2 | 4552-6 |
| 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.
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