Test Id : ADMAB
ADAMTS 13 Antibody, Plasma
Useful For
Suggests clinical disorders or settings where the test may be helpful
Assisting with the diagnosis and monitoring of congenital, immune, or acquired thrombotic thrombocytopenic purpura
Method Name
A short description of the method used to perform the test
Enzyme-Linked Immunosorbent Assay (ELISA)
NY State Available
Indicates the status of NY State approval and if the test is orderable for NY State clients.
Reporting Name
Lists a shorter or abbreviated version of the Published Name for a test
Aliases
Lists additional common names for a test, as an aid in searching
ADAMTS-13 Antibody
ADAMTS13 Antibody
ADMAB
aTTP
cTTP
iTTP
MAHA
microangiopathic hemolytic anemia
Microangiopathy
Thrombotic Microangiopathy
TTP
Specimen Type
Describes the specimen type validated for testing
Plasma Na Cit
Ordering Guidance
Consider ordering in patients to aid in distinguishing between congenital thrombotic thrombocytopenic purpura (TTP) and acquired autoimmune TTP.
Specimen Required
Defines the optimal specimen required to perform the test and the preferred volume to complete testing
Patient Preparation:
Fasting: 8 hours, preferred
Collection Container/Tube: Light-blue top (3.2% sodium citrate)
Submission Container/Tube: Polypropylene plastic vial
Specimen Volume: 1 mL plasma
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 into a separate plastic vial, 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.
Specimen Stability Information: Frozen 21 months
Additional Information:
1. Double-centrifuged specimen is critical for accurate 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.
1 mL
Reject Due To
Identifies specimen types and conditions that may cause the specimen to be rejected
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
Assisting with the diagnosis and monitoring of congenital, immune, or acquired thrombotic thrombocytopenic purpura
Clinical Information
Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test
Immune thrombotic thrombocytopenic purpura (iTTP) is a rare (estimated incidence of 3.7 cases per million) and potentially fatal thrombotic microangiopathy syndrome. It is characterized by thrombocytopenia and microangiopathic hemolytic anemia (intravascular hemolysis and presence of peripheral blood schistocytes), with no apparent alternative explanation of these findings. Other clinical features may include neurological symptoms, fever, and acute kidney injury. 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 is far more commonly acquired or immune mediated. iTTP 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 iTTP 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. Normally, 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 anti-ADAMTS13 IgG antibody interferes with this cleavage resulting in circulating ultra-high molecular weight multimers, microvascular occlusion, and leads to TTP.
The initial testing for diagnosis of TTP consists of testing for ADAMTS13 activity and, if indicated, assessment of an inhibitor against ADAMTS13. However, in the appropriate clinical circumstance, the decision to initiate plasma exchange should not be delayed pending results of this assay.
Approximately 35% of antibodies to ADAMTS13 are non-neutralizing. These will not be detected by a mixing study Bethesda type of assay but may foster enhanced clearance/reduction of ADAMTS13. Enzyme-linked immunosorbent assay ADAMTS13 antibody testing may be useful in determining congenital vs. immune TTP.(1)
Cohort studies have examined the prognostic impact of presence of ADAMTS13 IgG antibody in TTP. Although there are conflicting studies, the weight of evidence suggests that a low ADAMTS13 activity, an elevated Bethesda titer, presence of an IgG antibody and a low ADAMTS13 antigen are strong independent predictors of more severe disease, longer time to achieving remission and when present at remission a shorter time to relapse.
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.
Negative: <12 U/mL
Borderline positive: 12-15 U/mL
Positive: >15 U/mL
Interpretation
Provides information to assist in interpretation of the test results
Greater than or equal to 12 U/mL ADAMTS13 antibody is indicative of immune mediated thrombotic thrombocytopenic purpura in an appropriate clinical setting.
Cautions
Discusses conditions that may cause diagnostic confusion, including improper specimen collection and handling, inappropriate test selection, and interfering substances
Interferences of the ADAMTS13 antibody assay include high levels of hyperlipidemia. Lipemia can result in inconsistent to falsely elevated ADAMTS13 antibody U/mL, resulting in variable to false-positive test results using this methodology.
Recent treatment for thrombotic thrombocytopenic purpura (TTP) may reduce antibody levels; submission for testing prior to treatment is recommended.
Samples with high concentrations other than anti-ADAMTS13 autoantibodies may result in weak-positive or borderline-positive results.
Not all patients with a clinical diagnosis of TTP have a severe ADAMTS13 deficiency. Conversely, patients with other non-TTP conditions may have a moderate to severe ADAMTS13 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.
The impact of ADAMTS13 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. Dainese C, Valeri F, Bruno B, Borchiellini A. Anti-ADAMTS13 autoantibodies: From pathophysiology to prognostic impact-A review for clinicians. J Clin Med. 2023;12(17):5630. doi:10.3390/jcm12175630.
2. Nakashima MO, Zhang X, Rogers HJ, et al. Validation of a panel of ADAMTS13 assays for diagnosis of thrombotic thrombocytopenic purpura: activity, functional inhibitor, and autoantibody test. Int J Lab Hematol. 2016;38(5):550-559. doi:10.1111/ijlh.12542
3. Alwan F, Vendramin C, Vanhoorelbeke K, et al. Presenting ADAMTS13 antibody and antigen levels predict prognosis in immune-mediated thrombotic thrombocytopenic purpura. Blood. 2017;130(4):446-471. doi:10.1182/blood-2016-12-758656
4. Yang S, Jin M, Lin S, Cataland S, Wu H. ADAMTS13 activity and antigen during therapy and follow-up of patients with idiopathic thrombotic thrombocytopenic purpura: correlation with clinical outcome. Haematologica. 2011;96(10):1521-1527. doi:10.3324/haematol.2011.042945
5. Masias C, Cataland SR. The role of ADAMTS13 testing in the diagnosis and management of thrombotic microangiopathies and thrombosis. Blood. 2018;132(9):903-910. doi:10.1182/blood-2018-02-791533
6. Starke R, Machin S, Scully M, Purdy G, Mackie I. The clinical utility of ADAMTS13 activity, antigen and autoantibody assays in thrombotic thrombocytopenic purpura. Br J Haematol. 2007;136(4):649-655. doi:10.1111/j.1365-2141.2006.06471.x
7. MacArthur TA, Goswami J, Moon Tasson L, et al. Quantification of von Willebrand factor and ADAMTS-13 after traumatic injury: a pilot study. Trauma Surg Acute Care Open. 2021;6(1):e000703. doi:10.1136/tsaco-2021-000703
8. Edvardsen MS, Hansen ES, Ueland T, et al. Impact of the von Willebrand factor-ADAMTS-13 axis on the risk of future venous thromboembolism. J Thromb Haemost. 2023;21(5):1227-1237. doi:10.1016/j.jtha.2023.01.024
9. Rieger M, Mannucci PM, Kremer Hovinga JA, et al. ADAMTS13 autoantibodies in patients with thrombotic microangiopathies and other immunomediated diseases. Blood. 2005;106(4):1262-1267. doi:10.1182/blood-2004-11-4490
Method Description
Describes how the test is performed and provides a method-specific reference
Testing is performed on the Janus G3 liquid handler and BioTek microplate reader with Gen5 software using the Technoclone Technozym INH kit. The method used is an enzyme-linked immunosorbent assay.
A microtiter plate coated with a recombinant form of ADAMTS13 protease (rADAMTS13) in a 96 well polystyrene plate. Diluted patient plasma is incubated in the wells, allowing anti-ADAMTS-13 antibody in the sample to bind to the (rADAMTS13) in the wells. The plate is washed to remove unbound target. Conjugate: POX anti-human ADAMTS13 detection antibody is added to each well and incubated. After incubation, the conjugate is removed by washing. A chromogenic substrate of tetramethylbenzidine (TMB) is incubated and followed by a stopping solution of 2.5% sulfuric acid, to develop a color reaction. The intensity of the color is measured at 450 nm (reference of 620 nm) and is directly proportional to amount of ADAMTS13 antibody in the sample as determined against a reference line using assayed reference plasma.(Package insert: TECHNOZYM ADAMTS 13 INH ELISA, quantitative test for detection of human autoantibodies (IgG) in serum or plasma against ADAMTS13. Technoclone; Rev.023, 09/22/2021; Dekimpe C, Roose E, Kangro K, et al. Determination of anti-ADAMTS-13 autoantibody titers in ELISA: Influence of ADAMTS-13 presentation and autoantibody detection. J Thromb Haemost. 2021;19[9]:2248-2255. doi:10.1111/jth.15297)
PDF Report
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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.
Varies
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.
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
Indicates the location of the laboratory that performs the test
Fees :
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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.
CPT codes are provided by the performing laboratory.
83520
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 |
|---|---|---|
| ADMAB | ADAMTS 13 Antibody, P | 40824-5 |
| 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.
|
|---|---|---|
| 623136 | ADAMTS 13 Antibody | 40824-5 |
| 623272 | Interpretation | 69049-5 |