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ADAMTS13 Activity

Justification

Machaon provides the fastest ADAMTS13 activity and inhibitor testing available in the US. Rapid diagnosis and administration of correct treatment is paramount for TTP and aHUS. This test is diagnostic for Thrombotic Thrombocytopenic Purpura (TTP) when ADAMTS13 Activity levels are below 10%. Consider other Thrombotic Microangiopathies (TMAs), such as atypical Hemolytic Uremic Syndrome (aHUS), for ADAMTS13 Activity levels above 10%.

STAT: < 24 hours (7 days a week)

ELISA

Draw Tube: Blue Top

Sample Type: Citrated Plasma

DRAW KITS AVAILABLE

Specimen Requirements

Sample Type Volume Required Minimum Volume Stability
PREFERRED Citrated Plasma 1mL 0.5mL Room Temp.: 7 days*
Refrigerated: 7 days
Frozen (-20C): 2 weeks
Frozen (-80C): 6 months
ALTERNATIVE - - - -
REJECTION CRITERIA Thawed in transit if shipped frozen, refrozen, clotted sample
SPECIAL INSTRUCTIONS Mark Saturday delivery on FedEx air bill if shipping on a Friday.
*NY facilities must use Machaon's ADAMTS13 kits when shipping samples at room temp.

General Information

METHODOLOGY ELISA
STAT TAT < 24 hours (7 days a week)
STAT TAT Performance > 95% of results released in 15.5 hours 7 days a week
ROUTINE TAT < 24 hours (M-F)
ALTERNATIVE NAMES VWF-Cleaving Protease Activity, ADAMTS-13, cTTP diagnostic test, TTP diagnostic test
DESCRIPTION Chromogenic ELISA for the quantitative determination of ADAMTS13 activity in human plasma. ADAMTS13 is an enzyme that specifically cleaves large von Willebrand Factor multimers, which induce platelet thrombus formation under high shear stress. If the activity of ADAMTS13 is lowered for some reason, unusually large VWF multimers may accumulate.
LIMITATIONS Samples containing EDTA cannot be used because EDTA is a strong inhibitor of ADAMTS13 function. Hemolysis does not affect assay performance with samples containing up to 200mg/dL of hemoglobin. Lipemia does not affect assay performance with samples containing up to 300mg/dL of lipids. Icterus does not affect assay performance with samples containing up to 15mg/dL of bilirubin (both conjugated and unconjugated).
NORMAL RANGE 40-130%
ASSOCIATED TESTING ADAMTS13 Gene Sequencing; aHUS Genetic Panel; TMA-Complete Genetic Panel
REFERENCES

1. Moake, MD. Thrombotic microangiopathies. NEJM. 2002;347(8):589-600.
2. Tsai, HM. Autoimmune Thrombotic Microangiopathy: Advances in Pathogenesis, Diagnosis, and Management. Semin Thromb Hemost. 2012;38(05):469-482.
3. Sahud, M, Ero, M, et al. von Willebrand Factor-cleaving protease inhibitor in a patient with human immunodeficiency syndrome-associated thrombotic thrombocytopenic purpura. British Journal of Haematology. 2002;116:909-911.
SAMPLE REPORT Upon request
NEW YORK STATE APPROVED Yes

Test Codes

ORDER CODE P3336
CPT CODE 85397
LOINC CODE 53622-7