ADAMTS13 Activity with Reflex to Inhibitor

Overview

  • EPIC Code:
  • LAB14498
  • Soft Test Code:
  • ATS13
  • Send Out Test Code:
  • 145328
Alternate Names
  • ADAMTS13 Evaluation
  • von Willebrand Factor Protease Activity (ADAMTS-13
  • VWF Cleaving Protease (vWF-CP)
  • VWF Cleaving Protease (vWF-CP)(ADAMTS-13)
  • vWF Protease Activity (ADAMTS-13)
Clinical Significance

ADAMTS13 Activity with Reflex to Inhibitor [14532X] - 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


Specimen Collection & Preparation

Specimen Requirements

1.0 mL Frozen Platelet Poor Plasma from a Blue Top Tube - 3.2% Na Citrate in a Plastic Vial

Alternate Specimen

 

TransportAndStorage

Unacceptable Ambient
Unacceptable Refrigerated

21 days Frozen -20° (Transport)

Collection Instructions

Please submit a separate, frozen vial for each special coagulation assay ordered. 

Platelet-poor plasma:  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 ship on dry ice.

Please submit a separate, frozen vial for each special coagulation assay ordered.

Clinical Interpretation

Reference Range:

ADAMTS13 Activity 68-163 % Activity
ADAMTS13 Inhibitor <0.4 BEU


Test Comments:

If ADAMTS13 Activity is ≤30, then ADAMTS13 Inhibitor will be performed at an additional charge (CPT code(s): 85335)


Methodology:
  • Immunoassay (IA)

Clinical Significance

ADAMTS13 Activity with Reflex to Inhibitor [14532X] - 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


Production Schedule

Sites Performed
  • Quest - Chantilly
Days Performed
Sunday
Monday
Tuesday
Wednesday
Thursday
Friday
Departments
  • Sendouts - Clinical
Turn Around Time

2 to 5 days


Coding & Compliance

CDM

01428243; 00913883; 01428184; 00915335; 00913882; 00915397; 01238742


CPT Coding

85397