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ADAMTS13 Activity with Reflex to Inhibitor [14532X]
Test Code145328
Includes
If the ADAMTS13 Activity result is ≤0.30 IU/mL, then ADAMTS13 Inhibitor will be performed at an additional charge (CPT: 85335).
Preferred Specimen
1 mL frozen plasma collected in a 3.2% sodium citrate (light blue-top) tube
Minimum Volume
0.5 mL
Instructions
Please submit a separate, frozen vial for each special coagulation assay ordered. Draw blood in a light blue-top tube containing 3.2% sodium citrate, mix gently by inverting 3-4 times. Centrifuge 15 minutes at 1500 g within one hour of collection. Using a plastic pipette, remove plasma, taking care to avoid the WBC/platelet buffy layer and place into a plastic vial. Freeze immediately and transport on dry ice.
Transport Container
Transport tube
Transport Temperature
Frozen
Specimen Stability
Room temperature: Unacceptable
Refrigerated: Unacceptable
Frozen: 21 days
Refrigerated: Unacceptable
Frozen: 21 days
Reject Criteria (Eg, hemolysis? Lipemia? Thaw/Other?)
Gross hemolysis • Grossly lipemic • Grossly icteric
Methodology
Immunoassay (IA)
Setup Schedule
Set up: Sun-Fri; Report available: 2-4 days
Clinical Significance
ADAMTS-13 is a zinc metalloprotease that cleaves ultra large vWF multimers. Studies have shown that low levels of ADAMTS-13 activity are associated with thrombotic thrombocytopenic purpura (TTP), a life-threatening hematological condition characterized by a low platelet count, microvascular thrombi, red cell fragmentation, and renal complications. Congenital TTP is a rare inherited disease caused by mutations within the ADAMTS-13 gene, which result in the production of non-functional protein. The acquired form of TTP is an autoimmune-like disorder caused by the development of autoantibodies to ADAMTS-13 that inhibits enzyme activity.
