Search:
 
This image is of a spacer graphic
NHF Face Book NHF Twitter
+ Login to my NHF
+ NHF Membership
+ Donate to NHF
+ Chapter Center
+ Hechos y Respuestas Rápidas
+ Ethics Advisory Committee
This image is of a spacer graphic
-Learn About Coagulation Disorders
-What are Bleeding Disorders?
 History of Bleeding Disorders
-Types of Bleeding Disorders
 Hemophilia A
 Hemophilia B
 von Willebrand Disease
-Other Factor Deficiencies
 Factor I
 Factor II
 Factor V
-Factor VII
 Factor X
 Factor XI
 Factor XII
 Factor XIII
 Types of Bleeds
 Bleeding Disorders and Women
 Caring for the Newly Diagnosed Child
 Psychosocial Issues
 Complications, including Inhibitors
 Future Therapies
 What are Clotting Disorders?
 Comprehensive Medical Care - Hemophilia Treatment Centers
 Medical and Scientific Advisory Council
 Financial and Insurance Issues
 HANDI, NHF's Information Resource Center
 Resources
 Web Links
 Glossary

 

 

 
Factor VII Deficiency
 

(Can also be known as Alexander’s disease, stable factor deficiency, or proconvertin deficiency.  Not to be confused with acquired factor VII deficiency, which is associated with liver disease.)

Factor VII was first recognized in 1951, and originally named serum prothrombin version accerlerator (SPCA) deficiency.  Although the published incidence of Factor VII deficiency is estimated at 1 in 500,000, the disorder may be more common.  It is inherited in an autosomal recessive fashion, which means it affects men and women equally.  

The factor VII protein is part of the cascade of clotting factors that form the chain leading to a protective blood clot.  Factor VII deficiency is usually severe. In fact patients with less than 1% Factor VII activity experience similar symptoms to hemophilia.  People with severe factor VII are prone to joint bleeds.  In addition to spontaneous nosebleeds, people can experience bleeds in the stomach, intestines and urinary tract.  Head bleeds and muscle bleeds have also been reported.  Women can have severe menorrhagia.

Diagnosis is made through activated partial thromboplastin time (aPTT) test, prothrombin time (PT) test and thrombin time (TT) test.  Diagnosis can be confirmed with a factor VII assay.  There have been instances of combined Factor VII deficiencies with cases of Factors II, IX and X. 

Treatment

In July 2005, Novo Nordisk received FDA-approval for a new usage indication of its recombinant factor VIIa product Novoseven® to treat bleeding episodes in patients with factor VII deficiency.
 
Prothrombin complex concentrates (PCCs) can also be used to treat Factor VII deficiency.  However, the amount of factor VII contained in these products vary considerably among PCCs.  Not only is there a marked difference in factor content between the different commercial preparations, but factor content can also vary between product lots produced by the same manufacturer.  

Patients with factor VII deficiency can also be treated with fresh frozen plasma (FFP).
However, volume constraints may limit the amount of FFP that can be used. There have been cases of thrombosis reported in people with factor VII deficiency.

 

This section of our Web site is sponsored by: