Laboratory identification of factor inhibitors: the perspective of a large tertiary hemophilia center.
Coagulation factor inhibitors are antibodies that bind and neutralize specific procoagulant plasma proteins. The identification of coagulation factor inhibitors by the hemostasis laboratory requires a careful and systematic approach that excludes other possible causes of prolonged screening tests such as the activated partial thromboplastin time and prothrombin time. Once the laboratory is confident that a specific coagulation factor inhibitor is present in a sample, its strength or titer must be measured. The clinician will use this information as a treatment guide. The most frequently occurring factor inhibitors encountered in the hemostasis laboratory are those directed against factor VIII (FVIII), which can arise in individuals with inherited hemophilia A as an immune response to factor replacement therapy or as an autoantibody leading to the condition of acquired hemophilia A. The Bethesda assay is the most widely used test for measuring the FVIII inhibitor titer. The Bethesda assay has several components that must be carefully controlled to achieve consistent results. This overview examines the behavior of various coagulation inhibitors and laboratory tests with an emphasis on the Bethesda assay for factor inhibitors.
Kershaw
Geoffrey
G
Institute of Haematology, Royal Prince Alfred Hospital, Camperdown, Australia. geoffrey.kershaw@email.cs.nsw.gov.au
Jayakodi
Dayani
D
Dunkley
Scott
S
D000368:Aged; D019774:Blood Coagulation Factor Inhibitors / Q000032:analysis*; D001780:Blood Coagulation Tests / Q000379:methods*; D005164:Factor IX / Q000037:antagonists & inhibitors / Q000276:immunology; D005169:Factor VIII / Q000037:antagonists & inhibitors / Q000276:immunology*; D006467:Hemophilia A / Q000235:genetics / Q000276:immunology* / Q000503:physiopathology; D006801:Humans; D016682:Lupus Coagulation Inhibitor; D010314:Partial Thromboplastin Time / Q000379:methods / Q000592:standards; D011517:Prothrombin Time / Q000379:methods; D015203:Reproducibility of Results
D019774:Blood Coagulation Factor Inhibitors; D016682:Lupus Coagulation Inhibitor; D005169:Factor VIII; D005164:Factor IX