von Willebrand factor-
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.
What is an Adam 13 test?
Peyvandi F, Palla R, Lotta LA, et al. ADAMTS-13 assays in TTP. J Thromb Haemost 2010;8:631–40. 8. Rieger M, Mannucci PM, Kremer Hovinga JA, et al.
What causes ADAMTS13 deficiency?
ADAMTS13 deficiency can be acquired or congenital Secondary (23 to 67 percent of cases), arising from a variety of conditions, including autoimmune disorders, solid organ or hematopoietic cell transplant, malignancy, drugs and pregnancy.
Which syndrome is caused by the deficiency of plasma enzyme ADAMTS13?
Congenital or acquired deficiency of ADAMTS13 can result in thrombotic thrombocytopenic purpura (see Chapter 134).
What is ADAMTS13 inhibitor?
Interpretation. Less than 10% ADAMTS13 activity is highly indicative of thrombotic thrombocytopenic purpura (TTP) in an appropriate clinical setting. The presence of ADAMTS13 inhibition (positive inhibitor screen) with a measurable antibody titer is most consistent with an acquired TTP.
Why does vWF need to be cleaved?
ADAMTS13 helps prevent full vessel occlusion by cleaving large VWF fibrils into smaller structures that have reduced adhesive potential.
What is the function of VWF?
Von Willebrand factor (VWF) is a large multimeric glycoprotein that performs two critical functions in primary hemostasis: it acts as a bridging molecule at sites of vascular injury for normal platelet adhesion, and under high shear conditions, it promotes platelet aggregation.
How is ADAMTS13 deficiency treated?
Background: Therapeutic plasma exchange (TPE) is a proven treatment for thrombotic thrombocytopenic purpura (TTP) characterized by severe ADAMTS13 deficiency, but the efficacy of TPE in suspected TTP with an ADAMTS13 activity level of more than 10% remains controversial.
What is the normal range for ADAMTS13?
Normal plasma ADAMTS13 antigen levels span a concentration of 740-1420 ng/ml (median 1080 ng/ml) resulting in an ADAMTS13 activity to antigen ratio of 0.48 to 1.68 U/mug.
Which drugs can cause thrombocytopenia?
Heparin, a blood thinner, is the most common cause of drug-induced immune thrombocytopenia….Other medicines that cause drug-induced thrombocytopenia include:
- Furosemide.
- Gold, used to treat arthritis.
- Nonsteroidal anti-inflammatory drugs (NSAIDs)
- Penicillin.
- Quinidine.
- Quinine.
- Ranitidine.
- Sulfonamides.
Is ITP and TTP the same?
Are ITP and TTP the same thing? No, ITP and TTP are not the same thing. Both ITP and TTP are bleeding disorders, but they occur for different reasons and may require different treatments.
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.
What is the function of adadamts13?
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.
How does the ADAMTS13 enzyme work?
The ADAMTS13 enzyme cuts von Willebrand factor into smaller pieces to regulate its interaction with platelets. By processing von Willebrand factor in this way, the enzyme prevents it from triggering the formation of blood clots in normal circulation.
What is the role of ADAMTS13 in thrombosis?
Since the discovery of ADAMTS13, specific epitopes on its surface have been shown to be the target of inhibitory antibodies. Low levels of ADAMTS13 are also associated with an increased risk of arterial thrombosis, including myocardial infarction and cerebrovascular disease.
