Normal Function The ADAMTS13 enzyme processes a large protein called von Willebrand factor. This protein is involved in the first step of blood clotting at the site of injury, which is to help cells called platelets stick together and attach to the walls of blood vessels, forming temporary clots.
What is ADAMTS13 test?
ADAMTS13 Bethesda assays detect antibodies that neutralize function, which are present in approximately two-thirds of TTP cases. These are similar to the traditional Bethesda assays used to detect and titer coagulation factor VIII or IX inhibitors.
What is Upshaw Schulman syndrome?
Upshaw-Schulman syndrome (USS) is a congenital bleeding disorder characterized by repeated episodes of thrombocytopenia and microangiopathic hemolytic anemia that respond to infusions of fresh frozen plasma.
How long does an ADAMTS13 test take?
Tests
| Test Name | STAT Turnaround Time | Sample Requirement |
|---|---|---|
| ADAMTS13 Gene Sequencing | 48 hours (M-F, two consecutive business days required) | EDTA whole blood |
| ADAMTS13 Inhibitor | < 24 hours (7 days a week) | citrated plasma |
| Alpha-2 Antiplasmin Activity | < 1 week | citrated plasma |
| Anti-CFH Antibody | 48 hours | serum |
What is the history of ADAMTS13 deficiency?
Deficiency of ADAMTS13 was originally discovered in Upshaw Schulman Syndrome, the recurring familial form of thrombotic thrombocytopenic purpura. By that time it was already suspected that TTP occurred in the autoimmune form as well, owing to its response to plasmapheresis and characterisation of IgG inhibitors.
What is the role of ADAMTS13 in thrombocytopenic purpura?
Role in disease. Deficiency of ADAMTS13 was originally discovered in Upshaw Schulman Syndrome, the recurring familial form of thrombotic thrombocytopenic purpura. By that time it was already suspected that TTP occurred in the autoimmune form as well, owing to its response to plasmapheresis and characterisation of IgG inhibitors.
What does ADAMTS13 stand for?
ADAMTS13. ADAMTS13 ( a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 )—also known as von Willebrand factor-cleaving protease (VWFCP)—is a zinc -containing metalloprotease enzyme that cleaves von Willebrand factor (vWf), a large protein involved in blood clotting.
Why do autoantibodies neutralize ADAMTS13?
Autoantibodies neutralizing human ADAMTS13 (a disintegrin-like and metalloproteinase with thrombospondin type 1 motif), the metalloprotease that physiologically cleaves von Willebrand factor, are a major cause of severe deficiency of the protease and of acquired thrombotic thrombocytopenic purpura ( …
