Test Catalog

Test Id : AADAM

Not Orderable

ADAMTS13 Profile Interpretation

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Overview

Test Catalog

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

Technical interpretation of a profile to assist with the diagnosis of congenital, immune, or acquired thrombotic thrombocytopenic purpura including inhibitor studies

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

Only orderable as a reflex. For more information see ADAMP / ADAMTS13 Activity with Reflex Inhibitor Profile, Plasma

 

Technical 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

ADAMTS13 Profile Interpretation

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

Metalloprotease

Microangiopathy

Protease Activity Inhibitor for TTP

Thrombotic Microangiopathy

von Willebrand Factor Cleaving Protease

VWF protease activity and inhibitor

TTP

ADAMTS-13

ADM13

ADAMTS13

microangiopathic hemolytic anemia

MAHA

cTTP

iTTP

aTTP

Specimen Type
Describes the specimen type validated for testing

Plasma Na Cit

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

Only orderable as a reflex. For more information see ADAMP / ADAMTS13 Activity with Reflex Inhibitor Profile, Plasma

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

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

Technical interpretation of a profile to assist with the diagnosis of congenital, immune, or acquired thrombotic thrombocytopenic purpura including inhibitor studies

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 (TMA) 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 ADAMTS13 (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 ADAMTS13. ADAMTS13 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 ADAMTS13 activity and inhibition studies, the decision to initiate plasma exchange should not be delayed pending results of this assay.

 

ADAMTS13 and inhibitor Bethesda titer results can have an impact on overall survival, ultimate clinical outcome, responsiveness to plasma exchange, and relapse are still controversial in recent literature. Therefore, clinical correlation is essential.

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.

Only orderable as a reflex. For more information see ADAMP / ADAMTS13 Activity with Reflex Inhibitor Profile, Plasma

 

ADAMTS13 ACTIVITY ASSAY

> or =70%

ADMTS13 Inhibitor Titer

<0.5 BU

Interpretation
Provides information to assist in interpretation of the test results

Interpretive comments are provided.

 

Less than 10% ADAMTS13 activity is highly indicative of thrombotic thrombocytopenic purpura (TTP) in an appropriate clinical setting. The presence of ADAMTS-13 measurable Bethesda titer is most consistent with an acquired (autoimmune) TTP.

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

No significant cautionary statements

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

1. Sadler JE. Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura. Blood. 2008;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;116(20):4060-4069. doi:10.1182/blood-2010-07-271445

3. Upshaw JD. Congenital deficiency of a factor in normal plasma that reverses microangiopathic hemolysis and thrombocytopenia. N Engl J Med. 1978;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;2018(1):530-538. doi:10.1182/asheducation-2018.1.530

5. Mackie I, Mancini I, Muia J, et al. International Council for Standardization in Haematology (ICSH) recommendations for laboratory measurement of ADAMTS13. Int J Lab Hematol. 2020;42(6):685-696. doi:10.1111/ijlh.13295

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

Computer-generated interpretive comments will be provided based on the profile results.

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 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.

3 to 5 days

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

14 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.

Not Applicable

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
AADAM ADAMTS13 Profile Interpretation No LOINC Needed
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.
620818 ADAMTS13 Profile Interpretation 69049-5
620820 ADAMTS13 Profile Interpretation No LOINC Needed

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 | PHP Pdf | CMS 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