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{{Von Willebrand disease}}
{{Von Willebrand disease}}
{{CMG}}  {{AE}} {{PTD}}
{{CMG}}  {{AE}} {{PTD}} {{N.F}}


==Overview==
==Overview==
[[VWD]] is caused by a quantitative or qualitative defect in [[vWF]]. [[Von Willebrand Factor]] binds [[platelets]] to [[collagen]] and is important in primary [[hemostasis]].  It also serves as a carrier for [[Factor VIII]] and prevents it form being degraded.  Most cases of vWD are due to inherited mutations that affect production of vWF.  There are also acquired forms of vWD where vWF is impaired due to other pathological processes. Acquired defects in [[vWF]] can be caused by a number of conditions example [[mitral valve prolapse]], ventricular assist device, [[ventricular septal defect]], [[aortic stenosis]], monoclonal gammopathy of undetermined significance,  leukemia example [[chronic myeloid leukemia]] and [[chronic lymphocytic leukemia]], [[wilms tumor]], [[waldenström macroglobulinemia]],[[essential thrombocythemia]], [[multiple myeloma]], [[non-Hodgkin lymphoma]], [[polycythemia vera]], [[valproic acid]], [[ciprofloxacin]], [[griseofulvin]], [[systemic lupus erythematosus]],[[hypothyroidism]], [[uremia]], [[hemoglobinopathies]] and [[angiodysplasia]].<ref name="pmid4172730">{{cite journal| author=Simone JV, Cornet JA, Abildgaard CF| title=Acquired von Willebrand's syndrome in systemic lupus erythematosus. | journal=Blood | year= 1968 | volume= 31 | issue= 6 | pages= 806-12 | pmid=4172730 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4172730  }} </ref><ref name="pmid1083062">{{cite journal| author=Wautier JL, Levy-Toledano S, Caen JP| title=Acquired von Willebrand's syndrome and thrombopathy in a patient with chronic lymphocytic leukaemia. | journal=Scand J Haematol | year= 1976 | volume= 16 | issue= 2 | pages= 128-34 | pmid=1083062 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1083062  }} </ref><ref name="pmid17133419">{{cite journal| author=Franchini M, Lippi G| title=Acquired von Willebrand syndrome: an update. | journal=Am J Hematol | year= 2007 | volume= 82 | issue= 5 | pages= 368-75 | pmid=17133419 | doi=10.1002/ajh.20830 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17133419  }} </ref><ref name="pmid21540459">{{cite journal| author=Tiede A, Rand JH, Budde U, Ganser A, Federici AB| title=How I treat the acquired von Willebrand syndrome. | journal=Blood | year= 2011 | volume= 117 | issue= 25 | pages= 6777-85 | pmid=21540459 | doi=10.1182/blood-2010-11-297580 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21540459  }} </ref><ref name="pmid11838652">{{cite journal| author=Kumar S, Pruthi RK, Nichols WL| title=Acquired von Willebrand disease. | journal=Mayo Clin Proc | year= 2002 | volume= 77 | issue= 2 | pages= 181-7 | pmid=11838652 | doi=10.4065/77.2.181 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11838652  }} </ref><ref name="pmid10959686">{{cite journal| author=Veyradier A, Jenkins CS, Fressinaud E, Meyer D| title=Acquired von Willebrand syndrome: from pathophysiology to management. | journal=Thromb Haemost | year= 2000 | volume= 84 | issue= 2 | pages= 175-82 | pmid=10959686 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10959686  }} </ref><ref name="pmid10959711">{{cite journal| author=Federici AB, Rand JH, Bucciarelli P, Budde U, van Genderen PJ, Mohri H et al.| title=Acquired von Willebrand syndrome: data from an international registry. | journal=Thromb Haemost | year= 2000 | volume= 84 | issue= 2 | pages= 345-9 | pmid=10959711 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10959711  }} </ref>
[[VWD]] is caused by a quantitative or qualitative defect in [[vWF]]. Most cases of vWD are due to inherited mutations that affect production of vWF.  There are also acquired forms of vWD where vWF is impaired due to other pathological processes. Acquired defects in [[vWF]] can be caused by a number of conditions,for example [[mitral valve prolapse]], ventricular assist device, [[ventricular septal defect]], [[aortic stenosis]], [[monoclonal gammopathy]] of undetermined significance,  [[chronic myeloid leukemia]] and [[chronic lymphocytic leukemia]], [[wilms tumor]], [[waldenström macroglobulinemia]], [[essential thrombocythemia]], [[multiple myeloma]], [[non-Hodgkin lymphoma]], [[polycythemia vera]], [[valproic acid]], [[ciprofloxacin]], [[griseofulvin]], [[systemic lupus erythematosus]],[[hypothyroidism]], [[uremia]], [[hemoglobinopathies]] and [[angiodysplasia]].
 


==Causes==
==Causes==
[[VWD]] is caused by a quantitative or qualitative defect in [[vWF]].  [[Von Willebrand Factor]] binds [[platelets]] to [[collagen]] and is important in primary [[hemostasis]].  It also serves as a carrier for [[Factor VIII]] and prevents it form being degraded.  Most cases of vWD are due to inherited mutations that affect production of vWF.  There are also acquired forms of vWD where vWF is impaired due to other pathological processes. Acquired defects in [[vWF]] can be caused by a number of conditions including:<ref name="pmid4172730">{{cite journal| author=Simone JV, Cornet JA, Abildgaard CF| title=Acquired von Willebrand's syndrome in systemic lupus erythematosus. | journal=Blood | year= 1968 | volume= 31 | issue= 6 | pages= 806-12 | pmid=4172730 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4172730  }} </ref><ref name="pmid1083062">{{cite journal| author=Wautier JL, Levy-Toledano S, Caen JP| title=Acquired von Willebrand's syndrome and thrombopathy in a patient with chronic lymphocytic leukaemia. | journal=Scand J Haematol | year= 1976 | volume= 16 | issue= 2 | pages= 128-34 | pmid=1083062 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1083062  }} </ref><ref name="pmid17133419">{{cite journal| author=Franchini M, Lippi G| title=Acquired von Willebrand syndrome: an update. | journal=Am J Hematol | year= 2007 | volume= 82 | issue= 5 | pages= 368-75 | pmid=17133419 | doi=10.1002/ajh.20830 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17133419  }} </ref><ref name="pmid21540459">{{cite journal| author=Tiede A, Rand JH, Budde U, Ganser A, Federici AB| title=How I treat the acquired von Willebrand syndrome. | journal=Blood | year= 2011 | volume= 117 | issue= 25 | pages= 6777-85 | pmid=21540459 | doi=10.1182/blood-2010-11-297580 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21540459  }} </ref><ref name="pmid11838652">{{cite journal| author=Kumar S, Pruthi RK, Nichols WL| title=Acquired von Willebrand disease. | journal=Mayo Clin Proc | year= 2002 | volume= 77 | issue= 2 | pages= 181-7 | pmid=11838652 | doi=10.4065/77.2.181 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11838652  }} </ref><ref name="pmid10959686">{{cite journal| author=Veyradier A, Jenkins CS, Fressinaud E, Meyer D| title=Acquired von Willebrand syndrome: from pathophysiology to management. | journal=Thromb Haemost | year= 2000 | volume= 84 | issue= 2 | pages= 175-82 | pmid=10959686 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10959686  }} </ref><ref name="pmid10959711">{{cite journal| author=Federici AB, Rand JH, Bucciarelli P, Budde U, van Genderen PJ, Mohri H et al.| title=Acquired von Willebrand syndrome: data from an international registry. | journal=Thromb Haemost | year= 2000 | volume= 84 | issue= 2 | pages= 345-9 | pmid=10959711 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10959711  }} </ref>
===Life-threatening Causes===
 
*There are no life-threatening causes of von willebrand disease.
====Heart-related conditions====


===Common Causes===
Common causes of  von willebrand disease may include:<ref name="pmid4172730">{{cite journal| author=Simone JV, Cornet JA, Abildgaard CF| title=Acquired von Willebrand's syndrome in systemic lupus erythematosus. | journal=Blood | year= 1968 | volume= 31 | issue= 6 | pages= 806-12 | pmid=4172730 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4172730  }} </ref><ref name="pmid1083062">{{cite journal| author=Wautier JL, Levy-Toledano S, Caen JP| title=Acquired von Willebrand's syndrome and thrombopathy in a patient with chronic lymphocytic leukaemia. | journal=Scand J Haematol | year= 1976 | volume= 16 | issue= 2 | pages= 128-34 | pmid=1083062 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1083062  }} </ref>
*[[Chronic myeloid leukemia]]
*[[Chronic lymphocytic leukemia]]
*[[Wilms tumor]]
*[[Essential thrombocythemia]]
*[[Non-Hodgkin lymphoma]]
*[[Mitral valve prolapse]]
*[[Mitral valve prolapse]]
*Ventricular assist device
*Ventricular assist device
*[[Ventricular septal defect]]
*[[Ventricular septal defect]]
*[[Aortic stenosis]]
*[[Aortic stenosis]]
*[[Valproic acid]]
* [[Ciprofloxacin]]


====Malignant diseases====
===Less Common Causes===
*Monoclonal gammopathy of undetermined significance
Less common causes of von Willebrand disease  include:<ref name="pmid17133419">{{cite journal| author=Franchini M, Lippi G| title=Acquired von Willebrand syndrome: an update. | journal=Am J Hematol | year= 2007 | volume= 82 | issue= 5 | pages= 368-75 | pmid=17133419 | doi=10.1002/ajh.20830 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17133419  }} </ref>
 
*Leukemia example [[chronic myeloid leukemia]] and [[chronic lymphocytic leukemia]]
*[[Wilms tumor]]
*[[Waldenström macroglobulinemia]]
*[[Waldenström macroglobulinemia]]
*[[Essential thrombocythemia]]
*[[Multiple myeloma]]
*[[Multiple myeloma]]
*[[Non-Hodgkin lymphoma]]
*[[Polycythemia vera]]
*[[Polycythemia vera]]
*[[Wilms tumor]]
*[[Systemic lupus erythematosus]]
*[[Hypothyroidism]]
*[[Uremia]]
*[[Hemoglobinopathies]]
*[[Angiodysplasia]]


====Drugs and other agents====
===Genetic Causes===
*The vWF gene is located on the short arm ''p'' of chromosome 12 (12p13.2) von Willebrand disease is caused by a mutation in this gene.
 
===Causes by Organ System===
 
{| style="width:80%; height:100px" border="1"
| style="width:25%" bgcolor="LightSteelBlue" ; border="1" |'''Cardiovascular'''
| style="width:75%" bgcolor="Beige" ; border="1" |
*[[Mitral valve prolapse]]
*Ventricular assist device
*[[Ventricular septal defect]]
*[[Aortic stenosis]]
|-
| bgcolor="LightSteelBlue" | '''Chemical/Poisoning'''
| bgcolor="Beige" | No underlying causes
|-
|- bgcolor="LightSteelBlue"
| '''Dental'''
| bgcolor="Beige" | No underlying causes
|-
|- bgcolor="LightSteelBlue"
| '''Dermatologic'''
| bgcolor="Beige" | No underlying causes
|-
|- bgcolor="LightSteelBlue"
| '''Drug Side Effect'''
| bgcolor="Beige" |
*[[Valproic acid]]
*[[Valproic acid]]
* [[Ciprofloxacin]]
* [[Ciprofloxacin]]
*[[Griseofulvin]]
*[[Griseofulvin]]
|-
|- bgcolor="LightSteelBlue"
| '''Ear Nose Throat'''
| bgcolor="Beige" | No underlying causes
|-
|- bgcolor="LightSteelBlue"
| '''Endocrine'''
| bgcolor="Beige" | [[Hypothyroidism]]
|-
|- bgcolor="LightSteelBlue"
| '''Environmental'''
| bgcolor="Beige" | No underlying causes
|-
|- bgcolor="LightSteelBlue"
| '''Gastroenterologic'''
| bgcolor="Beige" | No underlying causes
|-
|- bgcolor="LightSteelBlue"
| '''Genetic'''
| bgcolor="Beige" |Mutations in vWF gene
|-
|- bgcolor="LightSteelBlue"
| '''Hematologic'''
| bgcolor="Beige" |
*[[Chronic myeloid leukemia]]
*[[Chronic lymphocytic leukemia]]
*[[Polycythemia vera]]
|-
|- bgcolor="LightSteelBlue"
| '''Iatrogenic'''
| bgcolor="Beige" | No underlying causes
|-
|- bgcolor="LightSteelBlue"
| '''Infectious Disease'''
| bgcolor="Beige" | No underlying causes
|-
|- bgcolor="LightSteelBlue"
| '''Musculoskeletal/Orthopedic'''
| bgcolor="Beige" | No underlying causes
|-
|- bgcolor="LightSteelBlue"
| '''Neurologic'''
| bgcolor="Beige" | No underlying causes
|-
|- bgcolor="LightSteelBlue"
| '''Nutritional/Metabolic'''
| bgcolor="Beige" | No underlying causes
|-
|- bgcolor="LightSteelBlue"
| '''Obstetric/Gynecologic'''
| bgcolor="Beige" | No underlying causes
|-
|- bgcolor="LightSteelBlue"
| '''Oncologic'''
| bgcolor="Beige" |
*[[Chronic myeloid leukemia]]
*[[Chronic lymphocytic leukemia]]
*[[Monoclonal gammopathy of undetermined significance]]
|-
|- bgcolor="LightSteelBlue"
| '''Ophthalmologic'''
| bgcolor="Beige" | No underlying causes
|-
|- bgcolor="LightSteelBlue"
| '''Overdose/Toxicity'''
| bgcolor="Beige" | No underlying causes
|-
|- bgcolor="LightSteelBlue"
| '''Psychiatric'''
| bgcolor="Beige" | No underlying causes
|-
|- bgcolor="LightSteelBlue"
| '''Pulmonary'''
| bgcolor="Beige" | No underlying causes
|-
|- bgcolor="LightSteelBlue"
| '''Renal/Electrolyte'''
| bgcolor="Beige" | [[Wilms tumor]]
|-
|- bgcolor="LightSteelBlue"
| '''Rheumatology/Immunology/Allergy'''
| bgcolor="Beige" |
* [[Systemic lupus erythematosus]]
* [[Waldenström macroglobulinemia]]
* [[Monoclonal gammopathy of undetermined significance]]
|-
|- bgcolor="LightSteelBlue"
| '''Sexual'''
| bgcolor="Beige" | No underlying causes
|-
|- bgcolor="LightSteelBlue"
| '''Trauma'''
| bgcolor="Beige" | No underlying causes
|-
|- bgcolor="LightSteelBlue"
| '''Urologic'''
| bgcolor="Beige" |[[Uremia]]
|-
|- bgcolor="LightSteelBlue"
| '''Miscellaneous'''
| bgcolor="Beige" | No underlying causes
|-
|}
===Causes in Alphabetical Order===
List the causes of the disease in alphabetical order:
<div style="-moz-column-count:3; column-count:3;">
* [[Aortic stenosis]]
* [[Angiodysplasia]]
* [[Ciprofloxacin]]
* [[Chronic myeloid leukemia]]
*[[Chronic lymphocytic leukemia]]
* [[Hypothyroidism]]
*[[Hemoglobinopathies]]
*[[Multiple myeloma]]
*[[Monoclonal gammopathy of undetermined significance]]
*[[Mitral valve prolapse]]
*[[Non-Hodgkin lymphoma]]
*[[Polycythemia vera]]
*[[Uremia]]
*Ventricular assist device
*[[Ventricular septal defect]]
*[[Valproic acid]]
*[[Wilms tumor]]
*[[Waldenström macroglobulinemia]]
*
*[[Waldenström macroglobulinemia]]


====Autoimmune disorders====
*[[Systemic lupus erythematosus]]


====Other disorders====
*[[Hypothyroidism]]
*[[Uremia]]
*[[Hemoglobinopathies]]
*[[Angiodysplasia]]


==References==
==References==

Latest revision as of 16:27, 28 September 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Prince Tano Djan, BSc, MBChB [2] Nazia Fuad M.D.

Overview

VWD is caused by a quantitative or qualitative defect in vWF. Most cases of vWD are due to inherited mutations that affect production of vWF. There are also acquired forms of vWD where vWF is impaired due to other pathological processes. Acquired defects in vWF can be caused by a number of conditions,for example mitral valve prolapse, ventricular assist device, ventricular septal defect, aortic stenosis, monoclonal gammopathy of undetermined significance, chronic myeloid leukemia and chronic lymphocytic leukemia, wilms tumor, waldenström macroglobulinemia, essential thrombocythemia, multiple myeloma, non-Hodgkin lymphoma, polycythemia vera, valproic acid, ciprofloxacin, griseofulvin, systemic lupus erythematosus,hypothyroidism, uremia, hemoglobinopathies and angiodysplasia.


Causes

Life-threatening Causes

  • There are no life-threatening causes of von willebrand disease.

Common Causes

Common causes of von willebrand disease may include:[1][2]

Less Common Causes

Less common causes of von Willebrand disease include:[3]

Genetic Causes

  • The vWF gene is located on the short arm p of chromosome 12 (12p13.2) von Willebrand disease is caused by a mutation in this gene.

Causes by Organ System

Cardiovascular
Chemical/Poisoning No underlying causes
Dental No underlying causes
Dermatologic No underlying causes
Drug Side Effect
Ear Nose Throat No underlying causes
Endocrine Hypothyroidism
Environmental No underlying causes
Gastroenterologic No underlying causes
Genetic Mutations in vWF gene
Hematologic
Iatrogenic No underlying causes
Infectious Disease No underlying causes
Musculoskeletal/Orthopedic No underlying causes
Neurologic No underlying causes
Nutritional/Metabolic No underlying causes
Obstetric/Gynecologic No underlying causes
Oncologic
Ophthalmologic No underlying causes
Overdose/Toxicity No underlying causes
Psychiatric No underlying causes
Pulmonary No underlying causes
Renal/Electrolyte Wilms tumor
Rheumatology/Immunology/Allergy
Sexual No underlying causes
Trauma No underlying causes
Urologic Uremia
Miscellaneous No underlying causes

Causes in Alphabetical Order

List the causes of the disease in alphabetical order:


References

  1. ↑ Simone JV, Cornet JA, Abildgaard CF (1968). "Acquired von Willebrand's syndrome in systemic lupus erythematosus". Blood. 31 (6): 806–12. PMID 4172730.
  2. ↑ Wautier JL, Levy-Toledano S, Caen JP (1976). "Acquired von Willebrand's syndrome and thrombopathy in a patient with chronic lymphocytic leukaemia". Scand J Haematol. 16 (2): 128–34. PMID 1083062.
  3. ↑ Franchini M, Lippi G (2007). "Acquired von Willebrand syndrome: an update". Am J Hematol. 82 (5): 368–75. doi:10.1002/ajh.20830. PMID 17133419.

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