- 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."
- "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."
- 1: Baillieres Clin Haematol 1998
- Acquired haemophilia.
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]