ADAMTS13 Activity and Inhibitor Profile Test Cost & Procedure

Von Willebrand factor (VWF) interacts with platelets and breaks down extremely large VWF multimers at the Tyr(1605)-Met(1606) bond in the A2 region of VWF under the control of a plasma protein known as ADAMTS13. Reduced or missing ADAMTS13 action can keep UL VWF in place because it can cause intravascular platelet buildup and microthrombi, which can cause clinical manifestations or arrows of thrombotic thrombocytopenic purpura.

TTP is a an occasional, life-threatening ailment that influences four to six patients per million populace. It is more common in women, especially pregnant or postpartum women (one case is estimated to occur in every 25,000 pregnancies), and in African-Americans. TTP is often diagnosed clinically, and getting the right diagnosis is frequently quite challenging. Microangiopathic hemolytic anaemia with plentiful schistocytes in the peripheral blood smudge, thrombocytopenia, neurologic manisfestation, fever, renal dismay, differed organ injury and ischemia, and downward statuses of ADAMTS13 action—typically small than 30%—are the primary factors of TTP. 

ADAMTS13 Activity and Inhibitor Profile.

Examination Code

ADM13

Contains

ADAMTS13 Activity and a response to the Bethesda Assay

Preferred Specimen: Two 4.5 mL vials of sodium citrate (blue tops). Consult the instructions for plasma specimens.

(1) 4.5 mL of sodium citrate is the minimum volume (blue tops). Consult the instructions for plasma specimens.

Instructions

Collection Guidelines for Plasma:

1. A sample needs to be taken before starting replacement therapy.
2. Spin plasma down, remove it, and spin plasma once more.
3. If at all possible, immediately freeze samples at -40 degrees Celsius.
4. The sample should be divided into two separate vials, each containing a minimum of 1 mL.
5. Ship samples together in the same box.

Other Details:

A double centrifuged specimen is necessary for accurate results because platelet contamination can produce false results.

• Citrate of sodium transport container (blue top)
• Carrying Temperature

Inpatient samples must be delivered right away to the lab.

Outpatient samples must be sent frozen (see instructions)

• Variety Stability

Temp in the room: unacceptable

Frozen: Not acceptable

2 weeks when frozen

Fluorescence resonance energy transfer is the method used (FRET)

Regarding Range

• > or =70% for ADAMTS13 Activity Assay
• Negative ADAMTS13 Inhibitor Screen
• 0.4 BU ADAMTS13 Bethesda Titer

Clinical Relevance

In a suitable clinical situation, 10% ADAMTS13 activity strongly suggests thrombotic thrombocytopenic purpura (TTP). The strongest evidence in favour of an acquired TTP is the presence of ADAMTS13 inhibition (positive inhibitor screen) with a quantifiable antibody titer.