234451005: Acquired von Willebrand disease (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).

\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).

\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).

\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).

Status: current, Sufficiently defined by necessary conditions definition status (core metadata concept). Date: 31-Jul 2018. Module: SNOMED CT core

Descriptions:

Id Description Lang Type Status Case? Module

5156397010 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). en Definition Active Entire term case sensitive (core metadata concept) SNOMED CT core

351278015 Acquired von Willebrand's disease en Synonym (core metadata concept) Active Only initial character case insensitive (core metadata concept) SNOMED CT core

1784908015 Acquired von Willebrand syndrome en Synonym (core metadata concept) Active Only initial character case insensitive (core metadata concept) SNOMED CT core

1784909011 Acquired von Willebrand disease en Synonym (core metadata concept) Active Only initial character case insensitive (core metadata concept) SNOMED CT core

5156395019 Acquired von Willebrand disease (disorder) en Fully specified name Active Only initial character case insensitive (core metadata concept) SNOMED CT core

3917121000005115 erhvervet von Willebrands sygdom da Synonym (core metadata concept) Active Only initial character case insensitive (core metadata concept) Danish module (core metadata concept)

Expanded Value Set

Outbound Relationships	Type	Target	Active	Characteristic	Refinability	Group	Values
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).	Is a	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.	true	Inferred relationship	Some
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).	Finding site	Entire hematological system (body structure)	false	Inferred relationship	Some
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).	Finding site	Body system structure	false	Inferred relationship	Some
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).	Has definitional manifestation	Hemostatic system finding	false	Inferred relationship	Some
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).	Occurrence	Any period of life commencing after birth, but before death.	true	Inferred relationship	Some	1
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).	Is a	Acquired coagulation disorder	true	Inferred relationship	Some
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).	Has interpretation	Abnormal	true	Inferred relationship	Some	2
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).	Interprets	Haemostatic function	true	Inferred relationship	Some	2

Inbound Relationships	Type	Active	Source	Characteristic	Refinability	Group
von Willebrand factor inhibitor disorder	Is a	True	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).	Inferred relationship	Some

This concept is not in any reference sets

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Id	Description	Lang	Type	Status	Case?	Module
5156397010	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).	en	Definition	Active	Entire term case sensitive (core metadata concept)	SNOMED CT core
351278015	Acquired von Willebrand's disease	en	Synonym (core metadata concept)	Active	Only initial character case insensitive (core metadata concept)	SNOMED CT core
1784908015	Acquired von Willebrand syndrome	en	Synonym (core metadata concept)	Active	Only initial character case insensitive (core metadata concept)	SNOMED CT core
1784909011	Acquired von Willebrand disease	en	Synonym (core metadata concept)	Active	Only initial character case insensitive (core metadata concept)	SNOMED CT core
5156395019	Acquired von Willebrand disease (disorder)	en	Fully specified name	Active	Only initial character case insensitive (core metadata concept)	SNOMED CT core
3917121000005115	erhvervet von Willebrands sygdom	da	Synonym (core metadata concept)	Active	Only initial character case insensitive (core metadata concept)	Danish module (core metadata concept)