| Primary Name | ADAMTS13 |
| Synonym | Von Willebrand factor cleaving protease |
| Synonym | Test for thrombotic thrombocytopenic purpura |
| Contraction | VWF cleaving protease |
| CPT Code | 85246, 85335 |
| Specimen | Collect two (2) blue-stopper tubes (3.2% sodium citrate), filled to specified volume. Specimen will be rejected if underfilled. |
| Specimen Management | Centrifuge blue-stopper tubes within one (1) hour of collection, separate plasma and test or quick-freeze at -70°C. |
| Specimen Accepted | Daily including weekends. Specimens from outside UAB Hospital will not be sent out to reference laboratories. |
| Test Performed | Assay is performed by reference laboratory. |
| Available Stat? | Not available stat as it is a sendout. Pathologist's approval required. |
| Description | ADAMTS13 is an enzyme of the family identified as "a disintegrin and metalloprotease with thrombospondin type-1 motifs." It's more common name is von Willebrand factor cleaving protease. The ADAMTS13 assay may be used to distinguish chronic recurring thrombotic thrombocytopenic purpura (TTP), TTP secondary to the presence of ADAMTS13 inhibitor, and hemolytic-uremic syndrome (HUS). |
| Reference Interval | ADAMTS13 is normally present in plasma. It is reduced in recurring TTP and in TTP due to the presence of an inhibitor, which may be demonstrated in the inhibitor portion of the assay. ADAMTS13 is present in HUS. |
| Associated With | Thrombotic thrombocytopenic purpura |
| Associated With | -- |
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