Table of ContentsView AllTable of ContentsPurpose of the TestRisks and ContraindicationsBefore the TestDuring the TestAfter the TestInterpreting Results
Table of ContentsView All
View All
Table of Contents
Purpose of the Test
Risks and Contraindications
Before the Test
During the Test
After the Test
Interpreting Results
Hemoglobin electrophoresis is a blood test commonly used to diagnose and characterize disorders of hemoglobin, called hemoglobinopathies.
There are several inherited hemoglobinopathies that can affect the ability of hemoglobin to perform its job normally.
The hemoglobin electrophoresis test is designed to detect genetic abnormalities in the structure of a person’s hemoglobin. Hemoglobin electrophoresis is typically done when a person has signs or symptoms ofhemolytic anemia, a family history of a hemoglobinopathy, an abnormalcomplete blood count (CBC)test, or a positive neonatal screening test.
Women of African, Mediterranean, Southeast Asian, West Indian, or Middle Eastern descent are at higher risk for hemoglobinopathies. The male partners of women who are found to have hemoglobinopathies should also have screening if they are considering having a child.
Verywell / JR Bee
The hemoglobin electrophoresis test is a blood test that can detect normal and abnormal hemoglobins, and begin to characterize the type of hemoglobinopathy if any exists. However, hemoglobin electrophoresis is only one of several tests that can detect and characterize abnormal hemoglobins.
Often, if an abnormal result is obtained with the electrophoresis test, sophisticated testing can be done to more precisely characterize hemoglobinopathies.
Different types of globin will move across the paper (or gel) at different speeds, and will thus separate themselves out into characteristic bands. By examining the bands that form during the application of the current, the types of hemoglobin present in the blood sample can be differentiated.
The hemoglobin electrophoresis test is a blood test. Consequently, there is almost no risk, aside from the small risk of bruising, bleeding, or infection that is present with any test requiring the drawing of blood.
The type of hemoglobin in your blood is not related to the time of day or what you have eaten or drunk lately, so there are no special instructions or restrictions you will need to follow before having a hemoglobin electrophoresis. The sample can be taken in any facility that performs standard blood drawing, at any time of day. Typically it is done in a healthcare provider’s office, a lab, or a hospital. As with any blood test, you should wear comfortable clothing with loose sleeves that can be pulled up easily to expose your arm.
The hemoglobin electrophoresis test is done with a standard blood draw. A tourniquet will be placed on your arm and a technician will feel for a suitable vein. Your skin will be cleaned with an alcohol wipe, and a needle will be inserted into the vein and the blood sample taken. After blood is drawn, a small bandage or gauze patch will be applied. You will then be allowed to go home.
Complications from a blood draw are extremely unusual. Keep an eye out for any further bleeding, bruising, or inflammation or infection. If bleeding should occur, place more pressure on the puncture site for 5 to 10 minutes, and if the problem persists, call your healthcare provider. You should also call your healthcare provider if you see signs of inflammation or infection (redness, tenderness, excessive pain, or swelling).
You should expect to hear the results of your hemoglobin electrophoresis test within a few days to a week. If your test is normal, it may be that that’s all you will hear.
However, you may get a more detailed report—or you may ask for a detailed report—even if the test is normal.
Normal Hemoglobin Values
In adults, normal values for hemoglobin molecules are given as percentages, as follows:
In children, higher levels of hemoglobin F are typical, with correspondingly lower levels of hemoglobin A and A2:
Abnormal Results
If you have any amount of an abnormal hemoglobin on your hemoglobin electrophoresis, you will need further evaluation.
Your healthcare provider will have to take into account many additional factors when interpreting the significance of the abnormal hemoglobin, including your family history, the results of your CBC (including particularly the hemoglobin,hematocrit, and themean corpuscular volume), the appearance of your red blood cells under the microscope, and the results of yourserum iron studies.
In addition, your healthcare provider may employ more sophisticated techniques to fully characterize and quantify the abnormal hemoglobin in your blood samples. Such testing may include high-pressure liquid chromatography, capillary zone electrophoresis, isoelectric focusing, or targeted genetic testing.
Understanding Hemoglobin and Hemoglobinopathies
Each hemoglobin molecule is a complex structure consisting of four protein subunits called globins, each of which is bound to a non-protein, iron-containing structure called a heme group. The four globin units in a hemoglobin molecule consist of two alpha-like and two beta-like chains.
Each globin unit carries a heme group comprised of a porphyrin ring and an iron ion. It is the job of the heme group to bind and carry oxygen, and to release it to the peripheral tissues at the right time. Each hemoglobin molecule can bind four oxygen molecules.
The newly deoxygenated hemoglobin in the peripheral tissues picks up some of the excess carbon dioxide it finds there and carries it back to the lungs. (Most of the waste carbon dioxide, however, reaches the lungs after it is dissolved in the blood.)
Hemoglobin accounts for the color of blood. Hemoglobin in the arteries, carrying plenty of oxygen, is bright red in color (which is how red blood cells got their name). Hemoglobin in the veins, having delivered its oxygen to the tissues, becomes more blueish in color.
Types of Normal Hemoglobin
In very early gestation, when the human embryo receives its oxygen from the yolk sac, embryonic hemoglobins are produced. The unique globin structures of embryonic hemoglobin allow for adequate oxygen exchange in the relatively low-oxygen environment of early fetal life.
Hemoglobinopathies
Numerous genetic mutations have been discovered that result in abnormalities of either the alpha-like or beta-like globins of the hemoglobin molecule. The abnormal hemoglobins resulting from these mutations are called hemoglobinopathies.
Over 1,000 kinds of hemoglobinopathies have been characterized so far. The majority of these are of minor significance and do not appear to cause clinical problems. They have been discovered, largely incidentally, in apparently normal people with the advent of screening hemoglobin electrophoresis tests.
However, several hemoglobinopathies do produce disease. The severity of a hemoglobinopathy usually depends on whether the mutation ishomozygous(inherited from both parents), or heterozygous (inherited from only one parent, with normal hemoglobin genes from the second parent). In general, with heterozygous hemoglobinopathies, enough “normal” hemoglobin is produced to mitigate to at least some degree any overall clinical manifestations. People with homozygous forms of hemoglobinopathy tend to have more severe clinical disease.
The hemoglobinopathies are generally divided into two categories:
Structural Hemoglobinopathies
Several structural hemoglobinopathies have been identified that produce clinical disease. Structural changes in the hemoglobin molecule can cause alterations in the shape and the flexibility of the red blood cells. The misshapen red cells can cause occlusion of blood vessels. Other kinds of structural hemoglobinopathies can cause hemolytic anemia. Yet other structural abnormalities can change the affinity of hemoglobin for oxygen.
Common structural hemoglobinopathies include:
Reduced Hemoglobin Production
Alpha thalassemias, most commonly seen in people of Asian or African descent, result in reduced alpha globin production.Beta thalassemias, most often seen in people of Mediterranean descent, cause reduced production of beta globin.
The thalassemias are genetically complex disorders, as several genetic mutations (alone or in combination) can produce thalassemia. The severity of thalassemia depends upon which globin chain is involved, and how many and which specific genes are causing the problem.
Combination Hemoglobinopathies
Occasionally, people inherit different hemoglobinopathy genes from each parent, resulting in what is called a compound heterozygous hemoglobinopathy or combination hemoglobinopathy. The more common combination hemoglobinopathies include:
Once the hemoglobinopathy has been fully characterized, you should expect your healthcare provider to have a detailed discussion with you over two topics: treatment you may need (if any), and genetic counseling.
If your hemoglobinopathy is the heterozygous form (so-called hemoglobin “trait,” in which you have inherited the abnormal hemoglobin from only one parent), between 45% to 65% of your hemoglobin very likely will be normal adult hemoglobin, and your symptoms, if any, are likely to be mild. Most people with hemoglobin traits do not require any specific treatment.
If you have a homozygous hemoglobinopathy, or a combination hemoglobinopathy (that is, two different abnormal hemoglobins), you may need treatment.
People with sickle cell disease today are almost always diagnosed in infancy with routine hemoglobin screening tests.These babies are treatedwith antibiotic prophylaxis, vitamin supplementation, full vaccination, and aggressive management of a sickle cell crisis whenever it occurs.
The thalassemias are a group of disorders whose effects vary widely depending on the specific genetic mutation that causes them. The most common problem they cause is anemia, but thalassemia can also cause skeletal abnormalities and iron overload as well as growth impairment and other disorders. People with severe thalassemia may require frequent blood transfusions and splenectomy.Iron overloadcan become a major problem in people with thalassemia.
Several uncommon hemoglobinopathies lead to “unstable hemoglobins,” where the structure of the hemoglobin molecules is altered in such a way to reduce the lifespan of the red blood cells. People with these conditions may experience anemia, enlarged spleens, and frequent infections. Treatment is aimed at preventing complications and may include blood transfusions, splenectomy, and avoiding oxidant drugs, including certain antibiotics and NSAIDs.Bone marrow transplantation is also being applied more often to people with severe, life-threatening hemoglobinopathies.
Genetic Counseling
11 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.American College of Obstetricians and Gynecologists.Carrier screening for genetic conditions. Committee opinion No. 691.Obstet Gynecol; 129:e41-55.Trent RJ.Diagnosis of the haemoglobinopathies.Clin Biochem Rev; 27:27. PMCID:PMC1390791American Association of Clinical Chemistry.Hemoglobinopathy evaluation.Hardison RC.Evolution of hemoglobin and its genes.Cold Spring Harb Perspect Med.2012; 2:a011627. doi:10.1101/cshperspect.a011627Brittain T.Molecular aspects of embryonic hemoglobin function.Mol Aspects Med.2002;23:293. doi:10.1016/s0098-2997(02)00004-3Kaufman DP, Lappin SL.Physiology, fetal hemoglobin. StatPearls.Forget BG, Bunn HF.Classification of the disorders of hemoglobin.Cold Spring Harb Perspect Med. 2013;3(2):a011684. doi:10.1101/cshperspect.a011684Martin A, Thompson AA.Thalassemias.Pediatr Clin North Am.2013;60:1383. doi:10.1016/j.pcl.2013.08.008de Montalembert M, Ferster A, Colombatti R, et al.ENERCA clinical recommendations for disease management and prevention of complications of sickle cell disease in children.Am J Hematol2011;86:72. doi:10.1002/ajh.21865Mishra AK, Tiwari A.Iron overload in beta thalassaemia major and intermedia patients.Maedica (Buchar); 8(4):328–332.Yates AM, Mortier NA, Hyde KS, Hankins, Ware RE.The diagnostic dilemma of congenital unstable hemoglobinopathies.Pediatr Blood Cancer2010;55:1393. doi:10.1002/pbc.22702
11 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.American College of Obstetricians and Gynecologists.Carrier screening for genetic conditions. Committee opinion No. 691.Obstet Gynecol; 129:e41-55.Trent RJ.Diagnosis of the haemoglobinopathies.Clin Biochem Rev; 27:27. PMCID:PMC1390791American Association of Clinical Chemistry.Hemoglobinopathy evaluation.Hardison RC.Evolution of hemoglobin and its genes.Cold Spring Harb Perspect Med.2012; 2:a011627. doi:10.1101/cshperspect.a011627Brittain T.Molecular aspects of embryonic hemoglobin function.Mol Aspects Med.2002;23:293. doi:10.1016/s0098-2997(02)00004-3Kaufman DP, Lappin SL.Physiology, fetal hemoglobin. StatPearls.Forget BG, Bunn HF.Classification of the disorders of hemoglobin.Cold Spring Harb Perspect Med. 2013;3(2):a011684. doi:10.1101/cshperspect.a011684Martin A, Thompson AA.Thalassemias.Pediatr Clin North Am.2013;60:1383. doi:10.1016/j.pcl.2013.08.008de Montalembert M, Ferster A, Colombatti R, et al.ENERCA clinical recommendations for disease management and prevention of complications of sickle cell disease in children.Am J Hematol2011;86:72. doi:10.1002/ajh.21865Mishra AK, Tiwari A.Iron overload in beta thalassaemia major and intermedia patients.Maedica (Buchar); 8(4):328–332.Yates AM, Mortier NA, Hyde KS, Hankins, Ware RE.The diagnostic dilemma of congenital unstable hemoglobinopathies.Pediatr Blood Cancer2010;55:1393. doi:10.1002/pbc.22702
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
American College of Obstetricians and Gynecologists.Carrier screening for genetic conditions. Committee opinion No. 691.Obstet Gynecol; 129:e41-55.Trent RJ.Diagnosis of the haemoglobinopathies.Clin Biochem Rev; 27:27. PMCID:PMC1390791American Association of Clinical Chemistry.Hemoglobinopathy evaluation.Hardison RC.Evolution of hemoglobin and its genes.Cold Spring Harb Perspect Med.2012; 2:a011627. doi:10.1101/cshperspect.a011627Brittain T.Molecular aspects of embryonic hemoglobin function.Mol Aspects Med.2002;23:293. doi:10.1016/s0098-2997(02)00004-3Kaufman DP, Lappin SL.Physiology, fetal hemoglobin. StatPearls.Forget BG, Bunn HF.Classification of the disorders of hemoglobin.Cold Spring Harb Perspect Med. 2013;3(2):a011684. doi:10.1101/cshperspect.a011684Martin A, Thompson AA.Thalassemias.Pediatr Clin North Am.2013;60:1383. doi:10.1016/j.pcl.2013.08.008de Montalembert M, Ferster A, Colombatti R, et al.ENERCA clinical recommendations for disease management and prevention of complications of sickle cell disease in children.Am J Hematol2011;86:72. doi:10.1002/ajh.21865Mishra AK, Tiwari A.Iron overload in beta thalassaemia major and intermedia patients.Maedica (Buchar); 8(4):328–332.Yates AM, Mortier NA, Hyde KS, Hankins, Ware RE.The diagnostic dilemma of congenital unstable hemoglobinopathies.Pediatr Blood Cancer2010;55:1393. doi:10.1002/pbc.22702
American College of Obstetricians and Gynecologists.Carrier screening for genetic conditions. Committee opinion No. 691.Obstet Gynecol; 129:e41-55.
Trent RJ.Diagnosis of the haemoglobinopathies.Clin Biochem Rev; 27:27. PMCID:PMC1390791
American Association of Clinical Chemistry.Hemoglobinopathy evaluation.
Hardison RC.Evolution of hemoglobin and its genes.Cold Spring Harb Perspect Med.2012; 2:a011627. doi:10.1101/cshperspect.a011627
Brittain T.Molecular aspects of embryonic hemoglobin function.Mol Aspects Med.2002;23:293. doi:10.1016/s0098-2997(02)00004-3
Kaufman DP, Lappin SL.Physiology, fetal hemoglobin. StatPearls.
Forget BG, Bunn HF.Classification of the disorders of hemoglobin.Cold Spring Harb Perspect Med. 2013;3(2):a011684. doi:10.1101/cshperspect.a011684
Martin A, Thompson AA.Thalassemias.Pediatr Clin North Am.2013;60:1383. doi:10.1016/j.pcl.2013.08.008
de Montalembert M, Ferster A, Colombatti R, et al.ENERCA clinical recommendations for disease management and prevention of complications of sickle cell disease in children.Am J Hematol2011;86:72. doi:10.1002/ajh.21865
Mishra AK, Tiwari A.Iron overload in beta thalassaemia major and intermedia patients.Maedica (Buchar); 8(4):328–332.
Yates AM, Mortier NA, Hyde KS, Hankins, Ware RE.The diagnostic dilemma of congenital unstable hemoglobinopathies.Pediatr Blood Cancer2010;55:1393. doi:10.1002/pbc.22702
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