Test Catalog

Test Id : HK1

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Hexokinase Enzyme Activity, Blood

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Overview

Test Catalog

Useful For
Suggests clinical disorders or settings where the test may be helpful

The evaluation of individuals with Coombs-negative chronic hemolysis

Method Name
A short description of the method used to perform the test

Kinetic Spectrophotometry (KS)

NY State Available
Indicates the status of NY State approval and if the test is orderable for NY State clients.

Yes

Reporting Name
Lists a shorter or abbreviated version of the Published Name for a test

Hexokinase, B

Specimen Type
Describes the specimen type validated for testing

Whole Blood ACD-B

Specimen Required
Defines the optimal specimen required to perform the test and the preferred volume to complete testing

Container/Tube:

Preferred: Yellow top (ACD solution B)

Acceptable: Lavender top (EDTA)

Specimen Volume: 6 mL

Collection Instructions: Send whole blood specimen in original tube. Do not aliquot.

Forms

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

Specimen Minimum Volume
Defines the amount of sample necessary to provide a clinically relevant result as determined by the testing laboratory. The minimum volume is sufficient for one attempt at testing.

1 mL

Reject Due To
Identifies specimen types and conditions that may cause the specimen to be rejected

Gross hemolysis Reject

Specimen Stability Information
Provides a description of the temperatures required to transport a specimen to the performing laboratory, alternate acceptable temperatures are also included

Specimen Type Temperature Time Special Container
Whole Blood ACD-B Refrigerated 20 days

Useful For
Suggests clinical disorders or settings where the test may be helpful

The evaluation of individuals with Coombs-negative chronic hemolysis

Clinical Information
Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test

Hexokinase (HK) is the first enzymatic step in glycolysis, converting glucose to glucose 6-phosphate. Hexokinase deficiency (OMIM 235700) is a rare cause of chronic nonspherocytic hemolytic anemia, and its inheritance is autosomal recessive. Clinically significant HK deficiency manifests in early onset anemia with variable severity ranging from mild to severe. Some patients show neurologic impairment of which the mechanism is unclear.

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.

> or = 12 months: 0.7-1.7 U/g Hb

Reference values have not been established for patients who are younger than12 months.

Interpretation
Provides information to assist in interpretation of the test results

Clinical correlation or genetic confirmation may be required to establish hexokinase (HK) deficiency as a cause of hemolytic anemia as the assayed activity level in confirmed cases can vary from markedly decreased to borderline normal levels due to a compensated increase in enzyme by reticulocytes. Comparison of HK activity levels to other RBC enzyme activity can be very useful. Heterozygotes have moderately decreased to low normal HK levels and are expected to be clinically unaffected. 

 

Increased HK activity may be seen when reticulocytes are increased and is not supportive of a diagnosis of HK deficiency.

Cautions
Discusses conditions that may cause diagnostic confusion, including improper specimen collection and handling, inappropriate test selection, and interfering substances

Recent transfusion may mask the patient's intrinsic enzyme activity and cause unreliable results.

 

Hexokinase (HK) activity level can vary from markedly decreased to borderline normal levels in affected individuals due to a compensated increase in enzyme by reticulocytes. Comparison of HK activity levels to other red blood cell enzyme activity can be very useful.

Clinical Reference
Recommendations for in-depth reading of a clinical nature

1. Koralkova P, Mojzikova R, van Oirschot B, et al: Molecular characterization of six new cases of red blood cell hexokinase deficiency yields four novel mutations in HK1. Blood Cells Mol Dis. 2016 Jul;59:71-76

2. Koralkova P, van Solinge WW, van Wijk R: Rare hereditary red blood cell enzymopathies associated with hemolytic anemia-pathophysiology, clinical aspects and laboratory diagnosis. Int J Lab Hematol. 2014;36:388-397

Method Description
Describes how the test is performed and provides a method-specific reference

Hexokinase catalyzes the reaction of adenosine triphosphate (ATP) and glucose to glucose 6-phosphate (G6P) and adenosine diphosphate (ADP). In this assay the formation of G6P is measured by linking its further oxidation to 6-phosphogluconate to the reduction of nicotinamide adenine dinucleotide phosphate (NADP[+]) through the G6P dehydrogenase reaction. The increase in absorbance, which occurs as NADP(+) is reduced to NADPH, is measured spectrophotometrically at 340 nm on an automated chemistry analyzer.(Beutler E: Red Cell Metabolism: A Manual of Biochemical Methods. 3rd ed. Grune and Stratton; 1984:40-42; van Solinge WW, van Wijk: Enzymes of the red blood cell. In: Rifai N, Horvath AR, Wittwer CT: eds. Tietz Textbook of Clinical Chemistry and Molecular Diagnostics. 6th ed. Elsevier; 2018:chap 30)

PDF Report
Indicates whether the report includes an additional document with charts, images or other enriched information

No

Day(s) Performed
Outlines the days the test is performed. This field reflects the day that the sample must be in the testing laboratory to begin the testing process and includes any specimen preparation and processing time before the test is performed. Some tests are listed as continuously performed, which means that assays are performed multiple times during the day.

Tuesday, Thursday

Report Available
The interval of time (receipt of sample at Mayo Clinic Laboratories to results available) taking into account standard setup days and weekends. The first day is the time that it typically takes for a result to be available. The last day is the time it might take, accounting for any necessary repeated testing.

1 to 6 days

Specimen Retention Time
Outlines the length of time after testing that a specimen is kept in the laboratory before it is discarded

7 days

Performing Laboratory Location
Indicates the location of the laboratory that performs the test

Rochester

Fees
Several factors determine the fee charged to perform a test. Contact your U.S. or International Regional Manager for information about establishing a fee schedule or to learn more about resources to optimize test selection.

  • Authorized users can sign in to Test Prices for detailed fee information.
  • Clients without access to Test Prices can contact Customer Service 24 hours a day, seven days a week.
  • Prospective clients should contact their account representative. For assistance, contact Customer Service.

Test Classification
Provides information regarding the medical device classification for laboratory test kits and reagents. Tests may be classified as cleared or approved by the US Food and Drug Administration (FDA) and used per manufacturer instructions, or as products that do not undergo full FDA review and approval, and are then labeled as an Analyte Specific Reagent (ASR) product.

This test was developed and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. It has not been cleared or approved by the US Food and Drug Administration.

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 Clinic Laboratories interpretation of CPT coding requirements. It is the responsibility of each laboratory to determine correct CPT codes to use for billing.

CPT codes are provided by the performing laboratory.

82657

LOINC® Information
Provides guidance in determining the Logical Observation Identifiers Names and Codes (LOINC) values for the order and results codes of this test. LOINC values are provided by the performing laboratory.

Test Id Test Order Name Order LOINC Value
HK1 Hexokinase, B 49216-5
Result Id Test Result Name Result LOINC Value
Applies only to results expressed in units of measure originally reported by the performing laboratory. These values do not apply to results that are converted to other units of measure.
HKCL Hexokinase, B 49216-5

Test Setup Resources

Setup Files
Test setup information contains test file definition details to support order and result interfacing between Mayo Clinic Laboratories and your Laboratory Information System.

Excel | Pdf

Sample Reports
Normal and Abnormal sample reports are provided as references for report appearance.

Normal Reports | Abnormal Reports

SI Sample Reports
International System (SI) of Unit reports are provided for a limited number of tests. These reports are intended for international account use and are only available through MayoLINK accounts that have been defined to receive them.

SI Normal Reports | SI Abnormal Reports