Table 6.

Practical points when testing thrombophilia

ThrombophiliaType of testPractical points
Inherited thrombophilia 
Factor V Leiden mutation Activated PC resistance screening testa Can be influenced by acute thrombosis, high estrogen states (pregnancy, hormone use), coagulation factor deficiencies (eg, FVIII, FIX), the presence of lupus anticoagulant, and anticoagulant use 
DNA test Can be tested regardless of use of anticoagulants or hormonal status 
Prothrombin G20210A mutation DNA test Can be tested regardless of use of anticoagulants or hormonal status 
PC deficiency Typically chromogenic, ELISA or functional clotting-based assay May be influenced by acute thrombosis, high estrogen states (pregnancy, hormone use), and anticoagulant use—interpret abnormal values with caution 
PS deficiency 
AT deficiency 
Acquired thrombophilia, antiphospholipid antibodiesb 
Lupus anticoagulant Substantial variation among laboratories; typically includes a screening (eg, dilute Russell viper venom test or lupus anticoagulant-sensitive prothrombin time) and a confirmation test (eg, mixing or correction test) Consult local laboratory for specifics, is generally influenced by anticoagulant use 
Anti−ß2-glycoprotein-I IgG and IgM antibodies Typically ELISA Substantial variation in absolute cutoff values, consult local laboratory for specifics 
Anticardiolipin IgG and IgM antibodies Typically ELISA Substantial variation in absolute cutoff values, consult local laboratory for specifics 
ThrombophiliaType of testPractical points
Inherited thrombophilia 
Factor V Leiden mutation Activated PC resistance screening testa Can be influenced by acute thrombosis, high estrogen states (pregnancy, hormone use), coagulation factor deficiencies (eg, FVIII, FIX), the presence of lupus anticoagulant, and anticoagulant use 
DNA test Can be tested regardless of use of anticoagulants or hormonal status 
Prothrombin G20210A mutation DNA test Can be tested regardless of use of anticoagulants or hormonal status 
PC deficiency Typically chromogenic, ELISA or functional clotting-based assay May be influenced by acute thrombosis, high estrogen states (pregnancy, hormone use), and anticoagulant use—interpret abnormal values with caution 
PS deficiency 
AT deficiency 
Acquired thrombophilia, antiphospholipid antibodiesb 
Lupus anticoagulant Substantial variation among laboratories; typically includes a screening (eg, dilute Russell viper venom test or lupus anticoagulant-sensitive prothrombin time) and a confirmation test (eg, mixing or correction test) Consult local laboratory for specifics, is generally influenced by anticoagulant use 
Anti−ß2-glycoprotein-I IgG and IgM antibodies Typically ELISA Substantial variation in absolute cutoff values, consult local laboratory for specifics 
Anticardiolipin IgG and IgM antibodies Typically ELISA Substantial variation in absolute cutoff values, consult local laboratory for specifics 
a

Activated PC resistance testing can be used as a screening test; confirmatory testing is done by DNA testing.

b

Also refer to guidance from the Scientific and Standardization Committee for lupus anticoagulant/antiphospholipid antibodies of the International Society on Thrombosis and Haemostasis and the 2023 American College of Rheumatology/European Alliance of Associations for Rheumatology Antiphospholipid Syndrome Classification Criteria.42,43 

ELISA, enzyme-linked immunosorbent assay; F, factor.

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