Version 2.78

Description

von Willebrand factor (vWF) cleaving protease, or ADAMTS13, is a metalloprotease that cleaves vWF and thereby limits platelet aggregation and formation of microthrombi. vWF cleaving protease inhibitors can decrease the protease activity and/or more rapidly remove the enzyme from the circulation. Such inhibitors are one cause of thrombotic thrombocytopenic purpura (TTP), a disorder characterized by thrombocytopenia, anemia, fever, and neurologic and renal abnormalities. Idiopathic TTP is often due to anti-ADAMTS13 autoantibodies, while inherited TTP is an autosomal recessive disorder due to mutations in the ADAMTS13 gene that lead to impaired protease function. Work-up of patients with TTP can include vWF cleaving protease activity and, if that activity is low, inhibitor screen followed by quantification. PMID: 18756543 Source: Regenstrief LOINC

Reference Information

Type Source Reference
Article New England Journal of MedicineCopyright Copyright © 2008 Massachusetts Medical Society Furlan, M, Robles, R, et al. von Willebrand Factor-Cleaving Protease in Thrombotic Thrombocytopenic Purpura and the Hemolytic-Uremic Syndrome. New England Journal Medicine. 1998 339:1578-1584. Link to New England Journal of Medicine
Article BloodCopyright Copyright © 2008 American Society of Hematology. M Furlan, M, Robles, Lamie, B. Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis. Blood. 1996;87:4223-4234. Link to Blood

Basic Part Properties

Part Display Name
von Willebrand factor (vWf) cleaving protease
Part Type
Component (Describes the core component or analyte measured)
Created On
2003-09-12
Construct for LOINC Short Name
vWF Cp

LOINC Terminology Service (API) using HL7® FHIR® Get Info

CodeSystem lookup
https://fhir.loinc.org/CodeSystem/$lookup?system=http://loinc.org&code=LP32458-9

Language Variants Get Info

Tag Language Translation
zh-CN Chinese (China) von Willebrand 因子裂解蛋白酶
Synonyms: von Willebrand 因子（vWf 因子、血管性假血友病因子、血管性血友病因子）裂解蛋白酶;ADAMTS13;von Willebrand 因子裂解蛋白酶（von Willebrand Factor Cleaving Protease，VWF-cp）;von Willebrand 因子裂解酶;vWCP;vWf 因子裂解蛋白酶;vWf 因子裂解酶;vWf 裂解酶;VWFCP;VWF-cp;血管性血友病因子裂解蛋白酶;血管性血友病因子裂解酶
fr-CA French (Canada) Protéase clivage du Facteur von Willebrand
es-ES Spanish (Spain) Proteasa divisora del Factor de von Willebrand
it-IT Italian (Italy) von Willebrand, proteasi di taglio fattore
Synonyms: Proteasi di taglio del fattore di von Willebrand
tr-TR Turkish (Turkey) von Willebrand faktörü parçalıyıcı proteaz
ru-RU Russian (Russian Federation) von Willebrand фактор (vWf) расщепляющая протеаза
nl-NL Dutch (Netherlands) von Willebrand-factor cleaving protease
fr-BE French (Belgium) Protéase de clivage du Facteur von Willebrand