Factor IX Inhibitor Evaluation
Clinical Information Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test
Factor IX inhibitors arise in patients with severe hemophilia B after factor IX transfusion. Patients with factor IX inhibitors may also develop anaphylactic reactions in response to factor IX infusions. Acquired factor IX inhibitors, occurring in previously healthy people, are exceedingly rare.
Detection and titering of coagulation inhibitor to the specific factor requested, primarily factor IX in patients with hemophilia B
Normally, there is no inhibitor (ie, negative result).
If the screening assays indicate the presence of an inhibitor, it will be quantitated and reported in Bethesda (or equivalent) units.
Cautions Discusses conditions that may cause diagnostic confusion, including improper specimen collection and handling, inappropriate test selection, and interfering substances
This test is not useful for detecting presence of inhibitors directed against other clotting factors and is not useful for the detection of a nonspecific circulating anticoagulant.
This assay will not detect presence of lupus anticoagulants.
If presence or type of inhibitor is unknown, PROCT / Prolonged Clotting Time or LUPPR / Lupus Anticoagulant Profile should be ordered.
Occasionally, a potent lupus-like anticoagulant may cause false-positive testing for a specific factor inhibitor (eg, factor VIII or IX). See preceding caution statement.
Reference Values Describes reference intervals and additional information for interpretation of test results. May include intervals based on age and sex when appropriate. Intervals are Mayo-derived, unless otherwise designated. If an interpretive report is provided, the reference value field will state this.
FACTOR IX ACTIVITY ASSAY
Normal, full-term newborn infants or healthy premature infants may have decreased levels (> or =20%), which may not reach adult levels for > or =180 days postnatal.*
*See Pediatric Hemostasis References in Coagulation Studies in Special Instructions.
FACTOR IX INHIBITOR SCREEN
Clinical References Provides recommendations for further in-depth reading of a clinical nature
1. Feinstein DI, Rapaport, SI: Acquired inhibitors of blood coagulation. In Hematology: Basic Principles and Practice. Edited by R Hoffman, EJ Benz Jr, SJ Shattil, et al. New York, Livingstone Press, 1991, pp 1380-1394
2. Chitlur M, Warrier I, Rajpurkar M, et al: Inhibitors in factor IX deficiency a report of the ISTH-SSC international FIX inhibitor registry (1997-2006). Haemophilia 2009;15(5):1027-1031