41106001: von Willebrand factor inhibitor disorder (disorder)

• SNOMED CT Concept\Clinical finding (finding)\...
• \Functional finding\Disorder of hemostatic system\Disorders involving the elements of blood coagulation, including platelets, coagulation factors and inhibitors, and the fibrinolytic system.\...
• \Acquired coagulation disorder\Acquired von Willebrand disease (VWD) is a rare bleeding disorder marked by the same biological anomalies as those seen in hereditary von Willebrand disease (VWD) but which occurs in association with another underlying pathology, generally in elderly patients without any personal or family history of bleeding anomalies. There are three principle pathogenic mechanisms described which are firstly the presence of autoantibodies (inhibiting or noninhibiting) that form immune complexes with the von Willebrand factor (VWF) leading to rapid clearance of VWF from the circulation. Secondly, absorption of VWF onto malignant cell clones and thirdly the increased proteolysis of high molecular weight VWF multimers under abnormal blood conditions caused by cardiovascular malformations (such as aortic valve stenosis).\von Willebrand factor inhibitor disorder
• \Acquired coagulation disorder\Acquired coagulation factor inhibitor disorder\Drug-induced coagulation inhibitor disorder (disorder)\von Willebrand factor inhibitor disorder
• \Includes true von Willebrand disease with mutation at the VWF locus, as well as mimicking disorders with other mutations (pseudo VWD) and acquired von Willebrand syndrome.\Acquired von Willebrand disease (VWD) is a rare bleeding disorder marked by the same biological anomalies as those seen in hereditary von Willebrand disease (VWD) but which occurs in association with another underlying pathology, generally in elderly patients without any personal or family history of bleeding anomalies. There are three principle pathogenic mechanisms described which are firstly the presence of autoantibodies (inhibiting or noninhibiting) that form immune complexes with the von Willebrand factor (VWF) leading to rapid clearance of VWF from the circulation. Secondly, absorption of VWF onto malignant cell clones and thirdly the increased proteolysis of high molecular weight VWF multimers under abnormal blood conditions caused by cardiovascular malformations (such as aortic valve stenosis).\von Willebrand factor inhibitor disorder
• \Disease\Disorder of hemostatic system\Disorders involving the elements of blood coagulation, including platelets, coagulation factors and inhibitors, and the fibrinolytic system.\...
• \Acquired coagulation disorder\Acquired von Willebrand disease (VWD) is a rare bleeding disorder marked by the same biological anomalies as those seen in hereditary von Willebrand disease (VWD) but which occurs in association with another underlying pathology, generally in elderly patients without any personal or family history of bleeding anomalies. There are three principle pathogenic mechanisms described which are firstly the presence of autoantibodies (inhibiting or noninhibiting) that form immune complexes with the von Willebrand factor (VWF) leading to rapid clearance of VWF from the circulation. Secondly, absorption of VWF onto malignant cell clones and thirdly the increased proteolysis of high molecular weight VWF multimers under abnormal blood conditions caused by cardiovascular malformations (such as aortic valve stenosis).\von Willebrand factor inhibitor disorder
• \Acquired coagulation disorder\Acquired coagulation factor inhibitor disorder\Drug-induced coagulation inhibitor disorder (disorder)\von Willebrand factor inhibitor disorder
• \Includes true von Willebrand disease with mutation at the VWF locus, as well as mimicking disorders with other mutations (pseudo VWD) and acquired von Willebrand syndrome.\Acquired von Willebrand disease (VWD) is a rare bleeding disorder marked by the same biological anomalies as those seen in hereditary von Willebrand disease (VWD) but which occurs in association with another underlying pathology, generally in elderly patients without any personal or family history of bleeding anomalies. There are three principle pathogenic mechanisms described which are firstly the presence of autoantibodies (inhibiting or noninhibiting) that form immune complexes with the von Willebrand factor (VWF) leading to rapid clearance of VWF from the circulation. Secondly, absorption of VWF onto malignant cell clones and thirdly the increased proteolysis of high molecular weight VWF multimers under abnormal blood conditions caused by cardiovascular malformations (such as aortic valve stenosis).\von Willebrand factor inhibitor disorder

Status: current, Not sufficiently defined by necessary conditions definition status (core metadata concept). Date: 31-Jan 2002. Module: SNOMED CT core

Descriptions:

Id	Description	Lang	Type	Status	Case?	Module
68571019	von Willebrand factor inhibitor disorder	en	Synonym (core metadata concept)	Active	Entire term case sensitive (core metadata concept)	SNOMED CT core
197546010	Acquired von Willebrand disease with autoantibodies	en	Synonym (core metadata concept)	Active	Only initial character case insensitive (core metadata concept)	SNOMED CT core
777858017	von Willebrand factor inhibitor disorder (disorder)	en	Fully specified name	Active	Entire term case sensitive (core metadata concept)	SNOMED CT core
1995761000005112	von Willebrands faktorinhibitorsygdom	da	Synonym (core metadata concept)	Active	Entire term case sensitive (core metadata concept)	Danish module (core metadata concept)

Expanded Value Set

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