Test Catalog

Test Id : 9INHE

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Factor IX Inhibitor Evaluation, Plasma

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Overview

Test Catalog

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

Detection and titering of coagulation inhibitor to the specific factor requested, primarily factor IX in patients with hemophilia B

 

This test is not useful for the detection of a lupus-like circulating anticoagulant inhibitor, a nonspecific circulating anticoagulant, or other inhibitors that are not specific for coagulation factors.

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
9INHT FIX Inhib Profile Tech Interp No Yes
F_9 Coag Factor IX Assay, P Yes Yes

Reflex Tests
Lists tests that may or may not be performed, at an additional charge, depending on the result and interpretation of the initial tests.

Test Id Reporting Name Available Separately Always Performed
9AINH FIX Inhib Profile Prof Interp No No
9BETH FIX Bethesda Units, P No No
F9_IS Factor IX Inhib Scrn No No

Testing Algorithm
Delineates situations when tests are added to the initial order. This includes reflex and additional tests.

Testing begins with coagulation factor IX activity assay with dilutions to evaluate assay inhibition; if the factor IX activity assay is normal or increased, then a technical interpretation will be provided.

 

If the factor IX activity assay is decreased, then an inhibitor screen will be performed at an additional charge to look for specific factor IX inhibition and a professional interpretation will be provided. If specific inhibition is apparent, the titer of the inhibitor will be determined.

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

F_9, 9BETH, F9_IS: Optical Clot-Based

9INHT: Technical Interpretation

9AINH: Medical Interpretation

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

Factor IX Inhib Profile, P

Aliases
Lists additional common names for a test, as an aid in searching

Factor IX Inhibitor Profile

Factor IX Inhibitor Screen, Plasma

Testing Algorithm
Delineates situations when tests are added to the initial order. This includes reflex and additional tests.

Testing begins with coagulation factor IX activity assay with dilutions to evaluate assay inhibition; if the factor IX activity assay is normal or increased, then a technical interpretation will be provided.

 

If the factor IX activity assay is decreased, then an inhibitor screen will be performed at an additional charge to look for specific factor IX inhibition and a professional interpretation will be provided. If specific inhibition is apparent, the titer of the inhibitor will be determined.

Specimen Type
Describes the specimen type validated for testing

Plasma Na Cit

Ordering Guidance

This test is for factor IX inhibitors only. If the patient is known to have hemophilia B, this is the correct test to order. If the presence or type of inhibitor is unknown, first order APROL / Prolonged Clot Time Profile, Plasma. When screening studies are needed for patients with known hemophilia A, order 8INHE / Factor VIII Inhibitor Evaluation, Plasma.

Shipping Instructions

Send all vials in the same shipping container.

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

Specimen Type: Platelet-poor plasma

Patient Preparation:

1. Patient must not be receiving Coumadin (warfarin) or heparin therapy

2. Fasting preferred

Collection Container/Tube: Light-blue top (3.2% sodium citrate)

Submission Container/Tube: Plastic vials

Specimen Volume: 3 mL in 3 plastic vials, each containing 1 mL

Collection Instructions:

1. Specimen must be collected prior to factor replacement therapy.

2. For complete instructions, see Coagulation Guidelines for Specimen Handling and Processing.

3. Centrifuge, transfer all plasma into a plastic vial, and centrifuge plasma again.

4. Aliquot plasma (1-2 mL per aliquot) into 3 separate plastic vials, leaving 0.25 mL in the bottom of centrifuged vial.

5. Freeze plasma immediately (no longer than 4 hours after collection) at -20 degrees C or, ideally, at -40 degrees C or below.

Additional Information:

1. Double-centrifuged specimen is critical for accurate results as platelet contamination may cause spurious results.

2. Each coagulation assay requested should have its own vial.

Special Instructions
Library of PDFs including pertinent information and forms related to the test

Forms

If not ordering electronically, complete, print, and send a Coagulation Test Request (T753) 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.

2 Plastic vials, each containing 1 mL

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

Gross hemolysis Reject
Gross lipemia Reject
Gross icterus 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
Plasma Na Cit Frozen 14 days

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

Detection and titering of coagulation inhibitor to the specific factor requested, primarily factor IX in patients with hemophilia B

 

This test is not useful for the detection of a lupus-like circulating anticoagulant inhibitor, a nonspecific circulating anticoagulant, or other inhibitors that are not specific for coagulation factors.

Testing Algorithm
Delineates situations when tests are added to the initial order. This includes reflex and additional tests.

Testing begins with coagulation factor IX activity assay with dilutions to evaluate assay inhibition; if the factor IX activity assay is normal or increased, then a technical interpretation will be provided.

 

If the factor IX activity assay is decreased, then an inhibitor screen will be performed at an additional charge to look for specific factor IX inhibition and a professional interpretation will be provided. If specific inhibition is apparent, the titer of the inhibitor will be determined.

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

Factor IX inhibitors arise in patients with severe hemophilia B after factor IX transfusion. Patients with factor IX inhibitors may also develop anaphylactic reactions in response to factor IX infusions. Acquired factor IX inhibitors, occurring in previously healthy people, are exceedingly rare.

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.

FACTOR IX ACTIVITY ASSAY

Adults: 65-140%

Normal, full-term newborn infants or healthy premature infants may have decreased levels (> or =20%) that may not reach adult levels for 180 days or more postnatal.*

*See Pediatric Hemostasis References section in Coagulation Guidelines for Specimen Handling and Processing.

 

FACTOR IX INHIBITOR SCREEN:

Negative

 

GENERAL FACTOR BETHESDA UNITS:

< or =0.4 Bethesda Units

Interpretation
Provides information to assist in interpretation of the test results

Normally, there is no inhibitor (ie, negative result).

 

If the screening assays indicate the presence of an inhibitor, it will be quantitated and reported in Bethesda (or equivalent) units.

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

Occasionally, a potent lupus-like anticoagulant may cause false-positive testing for a specific factor inhibitor (eg, factor VIII or IX).

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

1. Hoffman R, Benz Jr EJ, Silberstein LE, et al, eds. Hematology: Basic Principles and Practice. 7th ed. Elsevier; 2018

2. Chitlur M, Warrier I, Rajpurkar M, et al. Inhibitors in factor IX deficiency a report of the ISTH-SSC international FIX inhibitor registry (1997-2006). Haemophilia. 2009;15(5):1027-1031. doi:10.1111/j.1365-2516.2009.02039.x

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

This assay consists of measuring the difference in factor IX activity (partial thromboplastin time assay) before and after incubation of a mixture of normal plasma and patient's plasma for 1 hour at 37 degrees C. For optimal sensitivity, the factor IX value of the normal plasma is adjusted to approximately 20%, because the factor IX assay is more sensitive in this area of the curve. In addition, an excess of patient's plasma will make the test more sensitive to small amounts of inhibitors.(Owen CA Jr, Bowie EJW, Thompson JH Jr. The Diagnosis of Bleeding Disorders. 2nd ed. Little, Brown, and Company; 1975:143-145; Cielsa B. Defects of plasma clotting factors. In: Hematology in Practice. 3rd ed. FA Davis; 2019:chap 17)

 

If the inhibitor screen is positive for an inhibitor of factor IX, the inhibitor will be quantitated by the Bethesda assay. In the Bethesda procedure, inhibitors are quantified by mixing equal volumes of serially diluted plasma with normal plasma. This mixture is incubated 2 hours at 37 degrees C, and its factor IX activity is measured and compared to a control run at the same time. The difference between the factor IX activity of the patient's incubation mixture and that of the control is used to calculate the titer. The residual factor IX activity is converted to Bethesda units: 50% residual factor IX is equal to 1 Bethesda unit. Assays using the same basic principle as the Bethesda assay are used to quantitate the inhibitors of other coagulation factors.(Kasper CK, Aldedort LM, Counts RB, et al. A more uniform measurement of factor VIII inhibitors. Thromb Diath Haemorrh. 1975;34:869-872; Cielsa B. Defects of plasma clotting factors. In: Hematology in Practice. 3rd ed. FA Davis; 2019:chap 17)

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.

Monday through Friday

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 3 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 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
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.

85390

85250

85335 (if appropriate)

85335 (if appropriate)

85390 (if appropriate)

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
9INHE Factor IX Inhib Profile, P 96459-3
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.
F_9 Coag Factor IX Assay, P 3187-2
9INHT FIX Inhib Profile Tech Interp 69049-5

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