Inhibitors in factor IX deficiency a report of the ISTH-SSC international FIX inhibitor registry (1997-2006)

Meera Chitlur, I. Warrier, M. Rajpurkar, J. M. Lusher

Research output: Contribution to journalArticlepeer-review

111 Scopus citations

Abstract

Haemophilia B is an X-linked disorder resulting in coagulation factor IX deficiency. Patients with severe deficiency (<1% factor IX activity) may have significant bleeding complications similar to patients with haemophilia A or factor VIII deficiency. The development of inhibitory antibodies to the missing coagulation factor is a major complication in patients with haemophilia. While the incidence of inhibitors in patients with haemophilia A is higher than that in haemophilia B, the occurrence of allergic and or anaphylactic reactions with the development of inhibitors is unique to haemophilia B patients. Since haemophilia B is a rare bleeding disorder and the incidence of inhibitors is an even rarer entity, a registry was established by Dr Indira Warrier under the auspices of the FVIII/FIX subcommittee of the International Society of Thrombosis and Haemostasis, to gather information on the occurrence and characteristics of patients with inhibitors and also the incidence of allergic and anaphylactic reactions in this group of patients. This is the first report from this registry and helps us to gather some insight on haemophilia B patients with inhibitors and complications related to inhibitor development and difficulties with immune tolerance.

Original languageEnglish
Pages (from-to)1027-1031
Number of pages5
JournalHaemophilia
Volume15
Issue number5
DOIs
StatePublished - 2009

Keywords

  • Anaphylaxis
  • Haemophilia B
  • Immune tolerance
  • Inhibitor antibodies
  • Nephrotic syndrome

Fingerprint

Dive into the research topics of 'Inhibitors in factor IX deficiency a report of the ISTH-SSC international FIX inhibitor registry (1997-2006)'. Together they form a unique fingerprint.

Cite this