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hemophilia A |
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hemophilia A Classical hemophilia, factor VIII deficiency hemophilia Hematology An X-R coagulopathy due to a marked ↓ of factor VIII Physiology Factor VIII circulates as a noncovalent complex with von Willebrand factor, which once cleaved by thrombin or by factor Xa enables factor VIII to bind to phospholipid surfaces of damaged cells and adherent activated platelets Clinical Hemophilia A is heterogenous as moderate–1—4% normal; or mild–5—25% normal factor VIII deficiency occurs; classic findings–joint hemorrhage of knee > elbow >etc, muscle hemorrhage, bruising, prolonged and potentially fatal post-traumatic or post-op hemorrhage Lab Normal platelets, normal prothrombin time, ↑ aPTT, ↓ factor VIII Treatment Recombinant factor VIII. See AIDSgate, Factor VIII. How to thank TFD for its existence? Tell a friend about us, add a link to this page, add the site to iGoogle, or visit webmaster's page for free fun content. |
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| The most common form of the disease is hemophilia A, or classic hemophilia, in which the clotting factor VIII is either deficient or defective. Still, Selden admits to being "thrilled" with the results since this is the first gene therapy for hemophilia A in people. Syntonix is pursuing other early-stage programs, including a long-acting factor VIII program, with the potential to improve the treatment of hemophilia A. |
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