von Willebrand
Disease
This page is specific to 
Acquired von Willebrands Disease
Uploaded
22 Jul 2008

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

"Once in a while, people develop what appears to be von Willebrand disease later in life. When this occurs in those who have no family history of the disease, it is thought that they are probably producing antibodies that destroy or decrease the amount of von Willebrand factor. Some other people have "acquired" a form of the disease in association with another disorder, such as rheumatoid arthritis, systemic lupus erythematosus, kidney and certain cancers."
http://www.haemophilia.org.za/Vonwil2.htm
 
"Acquired vWD: Some individuals may develop vWD later in life. Acquired vWD is caused by antibodies that attack and destroy vWF. It is usually seen in patients with autoimmune disorders, such as systemic lupus erythematosus, rheumatoid arthritis or certain cancers. Moreover, drug-induced vWD (caused by drugs such as valproic acid and ciprofloxacin) occurs due to enhanced proteolysis of vWF."
http://www.uspharmacist.com/NewLook/Ce/von/lesson.cfm
 
1: Baillieres Clin Haematol 1998 Jun;11(2):287-303
Acquired haemophilia.
Hay CR.
Manchester Haemophilia Comprehensive Care Centre, University Department of Haematology, Manchester Royal Infirmary, UK.

Acquired haemophilia is a rare but life-threatening acquired bleeding diathesis caused by autoimmune depletion of factor VIII. This occurs most frequently in elderly patients who lack disease associations. Acquired haemophilia may also arise in association with SLE rheumatoid arthritis, Sjogren's syndrome, other autoimmune conditions, lymphoproliferative malignancy, pregnancy and as a drug reaction. Acquired haemophilia has an equal sex distribution. The aims of treatment are to eliminate the inhibitor by immunosuppression and to treat the bleeding, which is the most common cause of death in patients with acquired haemophilia. The inhibitor is abolished in up to 70% of patients using prednisolone and cyclophosphamide, although other immunosuppressive regimens may also be used. These include azathioprine, vincristine and other cytotoxic agents, high-dose immunoglobulin and cyclosporin A. Bleeding may be controlled using porcine factor VIII or recombinant factor VIIa, although human factor VIII and prothrombin complex concentrates also have a limited role as haemostatic agents in this condition.

PMID: 10097808 [PubMed - indexed for MEDLINE]
Links:
 
An 83-year-old with post-operative beeding
http://path.upmc.edu/cases/case285/dx.html
 
 
 
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