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factor V deficiency (Owren parahemophilia)
Etiology:
- inherited
- acquired inhibitor of factor V
- post partum
- treatment with a certain type of fibrin glue
- surgery
- autoimmune diseases
- certain cancers
Genetics:
- associated with defects in coagulation factor V
Clinical manifestations:
- similar to hemophilia, except bleeding into joints is less common
- bleeding into the skin
- bleeding of the gums
- excessive bruising
- excessive menstrual bleeding
- epistaxis
- prolonged or excessive loss of blood with surgery or trauma
- umbilical stump bleeding
Laboratory:
- factor V assay shows decreased activity
- thrombin time is normal
- partial thromboplastin time (PTT) is prolonged
- prothrombin time is prolonged
- bleeding time (slightly prolonged in some people)
Management:
- fresh frozen plasma for bleeding or after surgery
General
coagulation factor deficiency
Database Correlations
OMIM 227400
References
- OMIM :accession 227400
- Medline Plus: Factor V deficiency
http://www.nlm.nih.gov/medlineplus/ency/article/000550.htm