Von Willebrand disease causes: Difference between revisions
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==Overview== | ==Overview== | ||
[[VWD]] is caused by a quantitative or qualitative defect in [[vWF]]. | [[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]]. | [[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> | ||
====Heart-related conditions==== | ====Heart-related conditions==== | ||
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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]
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
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:[1][2][3][4][5][6][7]
- Mitral valve prolapse
- Ventricular assist device
- Ventricular septal defect
- Aortic stenosis
Malignant diseases
- 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
Drugs and other agents
Autoimmune disorders
Other disorders
References
- ↑ Simone JV, Cornet JA, Abildgaard CF (1968). "Acquired von Willebrand's syndrome in systemic lupus erythematosus". Blood. 31 (6): 806–12. PMID 4172730.
- ↑ 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.
- ↑ 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.
- ↑ Tiede A, Rand JH, Budde U, Ganser A, Federici AB (2011). "How I treat the acquired von Willebrand syndrome". Blood. 117 (25): 6777–85. doi:10.1182/blood-2010-11-297580. PMID 21540459.
- ↑ Kumar S, Pruthi RK, Nichols WL (2002). "Acquired von Willebrand disease". Mayo Clin Proc. 77 (2): 181–7. doi:10.4065/77.2.181. PMID 11838652.
- ↑ Veyradier A, Jenkins CS, Fressinaud E, Meyer D (2000). "Acquired von Willebrand syndrome: from pathophysiology to management". Thromb Haemost. 84 (2): 175–82. PMID 10959686.
- ↑ Federici AB, Rand JH, Bucciarelli P, Budde U, van Genderen PJ, Mohri H; et al. (2000). "Acquired von Willebrand syndrome: data from an international registry". Thromb Haemost. 84 (2): 345–9. PMID 10959711.