Inhibitors of propagation of coagulation (factors VIII, IX and XI): a review of current therapeutic practice.

Abstract

The management of patients with congenital haemophilia who develop alloantibodies against factors of the propagation phase of blood coagulation, commonly known as inhibitors, is the most important challenge facing haemophilia caregivers at present, as this complication not only compromises the efficacy of replacement therapy but also consumes an enormous amount of economic resources. Development of inhibitors further complicates the clinical course of severe haemophilia, with a prevalence of up to 30% in patients with haemophilia A (factor VIII deficiency) and up to 5% in those with haemophilia B (factor IX deficiency) and haemophilia C (factor XI deficiency). While the short-term goal of treatment of patients who develop alloantibodies is the control of bleeding, the eradication of the inhibitor is the main long-term goal. The management of severe bleeding episodes and the eradication of the autoantibody are also the mainstays of treatment of patients with acquired haemophilia, a rare but life-threatening haemorrhagic condition characterized by the development of inhibitory autoantibodies against coagulation factor VIII. The most recent options available for treating patients with congenital haemophilia complicated by inhibitors and acquired haemophilia because of autoantibodies against factor VIII are summarized in this review article.

• Authors: Massimo Franchini, Pier Mannuccio Mannucci
• Journal: British journal of clinical pharmacology (Br J Clin Pharmacol) Vol. 72 Issue 4 Pg. 553-62 (Oct 2011) ISSN: 1365-2125 [Electronic] England
• PMID: 21204915 (Publication Type: Journal Article, Review)
• Copyright: © 2011 The Authors. British Journal of Clinical Pharmacology © 2011 The British Pharmacological Society.

Chemical References

• Anticoagulants
• Blood Coagulation Factor Inhibitors
• Factor VIII
• Factor IX
• Factor XI

Topics

• Anticoagulants (therapeutic use)
• Blood Coagulation Factor Inhibitors (therapeutic use)
• Factor IX (antagonists & inhibitors, therapeutic use)
• Factor VIII (antagonists & inhibitors, therapeutic use)
• Factor XI (antagonists & inhibitors, therapeutic use)
• Hemophilia A (drug therapy)
• Hemophilia B (drug therapy)
• Humans