Test Catalog

Test Id : ADM13

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ADAMTS13 Activity and Inhibitor Profile, Plasma

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Overview

Test Catalog

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

Assisting with the diagnosis of congenital or acquired thrombotic thrombocytopenic purpura

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
ADMFX ADAMTS13 Activity Assay No Yes
ADMIN ADAMTS13 Interpretation No 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
ADMIS ADAMTS13 Inhibitor Screen No No
ADMBU ADAMTS13 Inhibitor Bethesda Titer No No

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

Testing begins with the ADAMTS-13 activity assay to evaluate the percent activity. If the ADAMTS-13 activity is less than 30%, an inhibitor screen will be performed to look for specific ADAMTS-13 inhibition. If specific inhibition is apparent, the titer of the inhibitor will be determined.

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

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

Fluorescence Resonance Energy Transfer (FRET)

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

ADAMTS13 Activity and Inhibitor Profile

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

Metalloprotease

Protease Activity Inhibitor for TTP

von Willebrand Factor Cleaving Protease

Microangiopathy

Thrombotic Microangiopathy

VWF protease activity and inhibitor

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

Testing begins with the ADAMTS-13 activity assay to evaluate the percent activity. If the ADAMTS-13 activity is less than 30%, an inhibitor screen will be performed to look for specific ADAMTS-13 inhibition. 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

Shipping Instructions

Send both vials in the same shipping container.

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

Patient Preparation: Fasting preferred

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

Submission Container/Tube: Plastic vials

Specimen Volume: 2 mL in 2 plastic vials each containing 1 mL

Collection Instructions:

1. Specimen must be collected prior to 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 mL per aliquot) into 2 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, below -40 degrees C.

Additional Information:

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

2. If priority specimen, mark request form, give reason, and request a call-back.

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

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

Forms

1. Coagulation Patient Information (T675)

2. If not ordering electronically, complete, print, and send 1 of the following forms with the specimen:

-Coagulation Test Request (T753)

-Renal Diagnostics Test Request (T830)

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 mL

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

  All specimens will be evaluated at Mayo Clinic Laboratories for test suitability.

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

Assisting with the diagnosis of congenital or acquired thrombotic thrombocytopenic purpura

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

Testing begins with the ADAMTS-13 activity assay to evaluate the percent activity. If the ADAMTS-13 activity is less than 30%, an inhibitor screen will be performed to look for specific ADAMTS-13 inhibition. 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

Thrombotic thrombocytopenic purpura (TTP), a rare (estimated incidence of 3.7 cases per million) and potentially fatal thrombotic microangiopathy syndrome, is characterized by a pentad of symptoms: thrombocytopenia, microangiopathic hemolytic anemia (intravascular hemolysis and presence of peripheral blood schistocytes), neurological symptoms, fever, and kidney dysfunction. A large majority of patients initially present with thrombocytopenia and peripheral blood evidence of microangiopathy and, in the absence of any other potential explanation for such findings, satisfy criteria for early initiation of plasma exchange, which is critical for patient survival. TTP may rarely be congenital (Upshaw-Shulman syndrome) but, far more commonly, is acquired. Acquired TTP may be considered primary or idiopathic (the most frequent type) or associated with distinctive clinical conditions (secondary TTP) such as medications, hematopoietic stem cell or solid organ transplantation, sepsis, and malignancy.

 

The isolation and characterization of an IgG autoantibody frequently found in patients with idiopathic TTP clarified the basis of this entity and led to the isolation and characterization of a metalloprotease called ADAMTS-13 (a disintegrin and metalloprotease with thrombospondin type 1 motif 13 repeats), which is the target for the IgG autoantibody, leading to a functional deficiency of ADAMTS-13. ADAMTS-13 cleaves the ultra-high-molecular-weight multimers of von Willebrand factor (VWF) at the peptide bond Tyr1605-Met1606 to disrupt VWF-induced platelet aggregation. The IgG antibody prevents this cleavage and leads to TTP. Although the diagnosis of TTP may be confirmed with ADAMTS-13 activity and inhibition studies, the decision to initiate plasma exchange should not be delayed pending results of this assay.

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.

ADAMTS13 ACTIVITY ASSAY

> or =70%

 

ADAMTS13 INHIBITOR SCREEN

Negative

 

ADAMTS13 BETHESDA TITER

<0.4 BU

Interpretation
Provides information to assist in interpretation of the test results

Less than 10% ADAMTS-13 activity is highly indicative of thrombotic thrombocytopenic purpura (TTP) in an appropriate clinical setting. The presence of ADAMTS-13 inhibition (positive inhibitor screen) with a measurable antibody titer is most consistent with an acquired TTP.

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

This ADAMTS-13 activity assay is an in vitro assay using a synthetic substrate peptide in a static liquid environment. The measured ADAMTS-13 activity may not reflect the true in vivo biological ADAMTS-13 activity.

 

Not all patients with a clinical diagnosis of idiopathic thrombotic thrombocytopenic purpura (TTP) have a severe ADAMTS-13 deficiency. Conversely, patients with other non-TTP conditions may have a severe ADAMTS-13 deficiency (< or =10%). These conditions include hemolytic uremic syndrome, hematopoietic stem cell and solid organ transplantation, liver disease, disseminated intravascular coagulation, sepsis, pregnancy, and certain medication. Therefore, TTP remains a clinical diagnosis.

 

Interferences of the ADAMTS-13 activity assay include high levels of endogenous von Willebrand factor, hyperlipidemia, hyperbilirubinemia (bilirubin concentration >30mg/dL), and cleavage by other proteases.

 

Recent plasma exchange or transfusion may falsely normalize ADAMTS-13 levels, thus potentially masking the diagnosis of TTP.

 

The impact of ADAMTS-13 levels and presence of inhibitors on overall survival, ultimate clinical outcome, responsiveness to plasma exchange, and relapse are still controversial. Therefore, clinical correlation is recommended.

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

1. Sadler JE: Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura. Blood. 2008 Jul 1;112(1):11-18. doi: 10.1182/blood-2008-02-078170

2. George JN: How I treat patients with thrombotic thrombocytopenic purpura: 2010. Blood. 2010 Nov 18;116(20):4060-4069. doi: 10.1182/blood-2010-07-271445

3. Upshaw JD Jr: Congenital deficiency of a factor in normal plasma that reverses microangiopathic hemolysis and thrombocytopenia. N Engl J Med. 1978 Jun 15;298(24):1350-1352. doi: 10.1056/NEJM197806152982407

4. Chiasakul T, Cuker A: Clinical and laboratory diagnosis of TTP: an integrated approach. Hematology Am Soc Hematol Educ Program. 2018 Nov 30;2018(1):530-538. doi: 10.1182/asheducation-2018.1.530

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

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

The ADAMTS-13 activity is measured by a fluorescence resonance energy transfer based assay using a synthetic fragment of von Willebrand factor as substrate and quantifying the cleavage of this small fragment by the ADAMTS-13 protease. The inhibitor screen and titer assay are performed using mixing studies that are similar to the Bethesda assay. One inhibitor (Bethesda) unit is defined as the concentration of an inhibitor that is able to reduce ADAMTS-13 activity of normal pooled plasma by 50%.(Mackie I, Mancini I, Muia J, Kremer Hovinga J, Nair S, Machin S, Baker R. International Council for Standardization in Haematology (ICSH) recommendations for laboratory measurement of ADAMTS13. Int J Lab Hematol. 2020 Dec;42(6):685-696. doi: 10.1111/ijlh.13295)

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, Sunday

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 was developed, and its performance characteristics 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.

85397

85335 (if appropriate)

85335 (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
ADM13 ADAMTS13 Activity and Inhibitor Profile 53622-7
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.
61211 ADAMTS13 Activity Assay 53622-7
34586 ADAMTS13 Interpretation 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