Test ID: HSEP
Hereditary Spherocytosis Evaluation
Secondary ID 
A test code used for billing and in test definitions created prior to November 2011
NY State Approved Indicates the status of NY State approval and if the test is orderable for NY State clients.
Useful For Suggests clinical disorders or settings where the test may be helpful
Investigation of suspected hereditary spherocytic hemolytic anemia
Profile Information A profile is a group of laboratory tests that are ordered and performed together under a single Mayo Test ID. Profile information lists the test performed, inclusive of the test fee, when a profile is ordered and includes reporting names and individual availability.
| Test ID | Reporting Name | Available Separately | Always Performed |
|---|---|---|---|
| FRAGO | Osmotic Fragility | No | Yes |
| SCTRL | Sex of Control Vial | No | Yes |
| HSEV | Spherocytosis Interpretation | No | Yes |
Reflex Tests Lists test(s) that may or may not be performed, at an additional charge, depending on the result and interpretation of the initial test(s)
| Test ID | Reporting Name | Available Separately | Always Performed |
|---|---|---|---|
| BND3 | Band 3 Fluorescence Staining, RBC | No | No |
Testing Algorithm Delineates situation(s) when tests are added to the initial order. This includes reflex and additional tests.
If the osmotic fragility test is abnormal, then the band 3 fluorescence staining will be performed at an additional charge.
Special Instructions and Forms Describes specimen collection and preparation information, test algorithms, and other information pertinent to test. Also includes pertinent information and consent forms to be used when requesting a particular test
Method Name A short description of the method used to perform the test
FRAGO/9064: Osmotic Lysis
BND3/83141: Flow Cytometry
Reporting Name A shorter/abbreviated version of the Published Name for a test; an abbreviated test name
Aliases Lists additional common names for a test, as an aid in searching
EMA Binding
Band 3
Specimen Type Describes the specimen type needed for testing
Whole Blood EDTA
Specimen Required Defines the optimal specimen. This field describes the type of specimen required to perform the test and the preferred volume to complete testing. The volume allows automated processing, fastest throughput and, when indicated, repeat or reflex testing.
See Recommendations for Collection of Control Specimens for FRAG, HSEP, and HAEVP in Special Instructions.
Forms: If not ordering electronically, please submit a Hematopathology/Molecular Oncology Request Form (Supply T241) with the specimen.
Specimens must arrive in the testing laboratory within 96 hours of draw.
A whole blood EDTA specimen and a control specimen are required.
Patient:
Container/Tube: Lavender top (EDTA)
Specimen Volume: 5 mL
Collection Instructions:
1. Immediately refrigerate specimen after draw.
2. Do not transfer blood to other containers.
3. Rubber band patient specimen and control vial together.
Additional Information: Patient's age and sex are required.
Normal Control:
Specimen Type: Whole blood
Container/Tube: Lavender top (EDTA)
Specimen Volume: 5 mL
Collection Instructions:
1. Draw a control specimen at the same time from a normal, unrelated, non-smoking individual.
2. Clearly write normal control on outermost label and also indicate sex of control on same label.
3. Immediately refrigerate specimen after draw.
4. Do not transfer blood to other containers.
5. Rubber band control vial and patient specimen together.
Specimen Minimum Volume Defines the amount of specimen required to perform an assay once, including instrument and container dead space. Submitting the minimum specimen volume makes it impossible to repeat the test or perform confirmatory or perform reflex testing. In some situations, a minimum specimen volume may result in a QNS (quantity not sufficient) result, requiring a second specimen to be collected.
Reject Due To Identifies specimen types and conditions that may cause the specimen to be rejected
| Hemolysis | Mild OK; Gross reject |
| Lipemia | NA |
| Icterus | NA |
| Other | NA |
Specimen Stability Information Provides a description of the temperatures required to transport a specimen to the laboratory. Alternate acceptable temperature(s) are also included.
| Specimen Type | Temperature | Time |
|---|---|---|
| Control | Refrigerated | 4 days |
| Whole Blood EDTA | Refrigerated | 4 days |
Clinical Information Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test
The hemolytic anemias are a group of anemias that are characterized by an increased destruction of RBCs. Anemias may be divided into inherited or acquired. Hereditary spherocytosis (HS), also known as congenital hemolytic anemia, is inherited as a non sex-linked dominant trait. HS is caused by a RBC membrane defect. The RBCs are spherocytic in shape and show an increased rate of destruction. HS can result from abnormalities involving several red cell membrane proteins, such as band 3, spectrin, and ankyrin.
Most often HS is diagnosed in childhood, adolescence, or early adult life. The diagnosis of HS is usually made by a combination of patient and family history, laboratory evidence of hemolysis, and review of a peripheral blood smear. The osmotic fragility test is usually markedly abnormal in these cases. However, factors such as age, sex, and medications can affect the osmotic fragility test.
This evaluation combines osmotic fragility testing with a newly developed flow cytometry assay to provide complementary information in the evaluation of patients with suspected HS.
Reference Values Describes reference intervals and additional information for interpretation of test results. May include intervals based on age and sex when appropriate. Intervals are Mayo-derived, unless otherwise designated. If an interpretive report is provided, the reference value field will state this.
0.50 g/dL NaCl (unincubated)
Males: 0.0-47.8% hemolysis
Females: 0.0-31.1% hemolysis
0.60 g/dL NaCl (incubated)
Males: 18.7-67.4% hemolysis
Females: 10.9-65.5% hemolysis
0.65 g/dL NaCl (incubated)
Males: 4.4-36.6% hemolysis
Females: 0.2-39.3% hemolysis
0.75 g/dL NaCl (incubated)
Males: 0.8-9.1% hemolysis
Females: 0.0-10.9% hemolysis
An interpretive report will be provided.
Interpretation Provides information to assist in interpretation of the test results
An interpretive report will be provided.
Cautions Discusses conditions that may cause diagnostic confusion, including improper specimen collection and handling, inappropriate test selection, and interfering substances
Interfering factors:
-Recent transfusion
-Oral contraceptives
-H2 blockers
Clinical Reference Provides recommendations for further in-depth reading of a clinical nature
1. Gladen BE, Luicens JN: Hereditary spherocytosis and other anemias due to abnormalities of the red cell membrane. In Wintrobe's Clinical Hematology. 10th edition. Edited by GR Lee, J Foerester, J Lukens, et al. Baltimore, Williams and Wilkins, 1999 pp 1132-1159
2. Gallagher PG, Jarolim P: Red cell membrane disorders. In Hematology: Basic Principles and Practice. Third edition. Edited by R Hoffman, EJ Benz, SJ Shattil, et al: New York, Churchill Livingstone, 2000 pp 576-610
Method Description Describes how the test is performed and provides a method-specific reference
FRAG/9064 Osmotic Fragility, Erythrocytes:
Specimens for erythrocyte osmotic fragility tests are anticoagulated with EDTA. Osmotic lysis is performed using sodium chloride (NaCl) solution, 0.50 g/dL. An incubated fragility test is performed following 24-hour incubation at 37 degrees C at the following NaCl concentrations: 0.60, 0.65, and 0.75 g/dL. Results are reported and interpreted.(Larson CJ, Scheidt R, Fairbanks VF: The osmotic fragility test for hereditary spherocytosis: use of EDTA-anticoagulated blood stored at 4 degrees C for up to 96 hours. Am Soc Clin Pathol Meeting Abstract, 1988; Larson CJ, Scheidt R, Fairbanks VF: The osmotic fragility test for hereditary spherocytosis: objective criteria for test interpretation. Am Soc Clin Pathol Meeting Abstract, 1988)
Band 3:
Eosin-5-malemide (EMA) is a fluorescent dye that binds to Lys-430 of the extracellular loop of the band 3 protein. Using a 1-color flow cytometry method (number of events plotted against fluorescence), the fluorescent intensity of EMA-stained RBC, is assessed and compared to normal-value patients.(King MJ, Behrens J, Rogers C, et al: Rapid flow cytometric test for the diagnosis of membrane cytoskeletal associated hemolytic anemia. Br J Haematol 2000;111:924-933)
Day(s) and Time(s) Test Performed Outlines the days and times the test is performed. This field reflects the day and time the sample must be in the testing laboratory to begin the testing process and includes any specimen preparation and processing time required before the test is performed. Some tests are listed as continuously performed, which means assays are performed several times during the day.
Monday through Saturday; varies
Analytic Time Defines the amount of time it takes the laboratory to setup and perform the test. This is defined in number of days. The shortest interval of time expressed is "same day/1 day," which means the results may be available the same day that the sample is received in the testing laboratory. One day means results are available 1 day after the sample is received in the laboratory.
Maximum Laboratory Time Defines the maximum time from specimen receipt at Mayo Medical Laboratories until the release of the test result
Specimen Retention Time Outlines the length of time after testing that a specimen is kept in the laboratory before it is discarded
Performing Laboratory Location The location of the laboratory that performs the test
CPT Code Information Provides guidance in determining the appropriate Current Procedural Terminology (CPT) code(s) information for each test or profile. The listed CPT codes reflect Mayo Medical Laboratories interpretation of CPT coding requirements. It is the responsibility of each laboratory to determine correct CPT codes to use for billing.
85557-Osmotic fragility
88184-Band 3 by flow cytometry (if appropriate)
LOINC® Code Information Provides guidance in determining the Logical Observation Identifiers Names and Codes (LOINC) values for the result codes returned for this test or profile.
| Result ID | Reporting Name | LOINC Code |
|---|---|---|
| 9064 | Osmotic Fragility, RBC | 34964-7 |
| SCTRL | Sex of Control Vial | N/A |
| 13065 | Spherocytosis Interpretation | In Process |
| 3306 | Osmotic Fragility, 0.50 g/dL NaCl | 23915-2 |
| 3307 | Osmotic Fragility, 0.60 g/dL NaCl | 23917-8 |
| 3308 | Osmotic Fragility, 0.65 g/dL NaCl | 23919-4 |
| 3309 | Osmotic Fragility, 0.75 g/dL NaCl | 30543-3 |
| 3310 | Osmotic Fragility Comment | In Process |
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