| 5. Specialized Tests for Bleeding Disorders |
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5.1: Refer to the ISTH SCC for recommendations for all specialized hemostasis testing for all inherited and acquired bleeding disorders.
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5.2: Formulate follow-up testing as appropriate based on the results of specialized testing for bleeding disorders.
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5.3: Perform appropriate and validated mixing studies in patients with a prolonged PT, APTT, and/or TT to distinguish between factor deficient state or the presence of an inhibitor
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5.4: Carry out appropriate tests to distinguish between a specific or non-specific inhibitor (e.g. Lupus Anticoagulant) and quantify a Bethesda titre.
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5.5: Investigate for specific factor deficiencies using a systematic approach that takes into consideration reagent factor and Lupus Anticoagulant sensitivities (e.g. elevated APTT, normal PT).
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5.6: Apply understanding of the difference between one-stage and chromogenic (or two-stage) factor (F) VIII and FIX assays in the diagnosis and monitoring of patients with a deficiency state and substitution therapies.
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5.7 Determine activity and antigen levels in the setting of fibrinogen or FII deficiency
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5.8 Test for rare bleeding disorders and abnormalities not detected during screening (eg. FXIII deficiency, FXI deficiency/Hemophilia C or fibrinolytic abnormalities in patients with clinically significant bleeding
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5.9 Perform and interpret comprehensive qualitative and quantitative testing for VWD, including antigen and activity analysis (e.g. Ristoncetin-dependent and independent function von Willebrand factor assays)
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5.10 Perform appropriate aggregation mixing studies to differentiate between type 2B VWD and platelet type VWD
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5.11 Assess platelet function (aggregation, secretion, flow cytometry analysis)
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5.12 Carry-out appropriate tests for the monitoring of platelet inhibitor therapies
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5.13 Apply understanding of genetic testing in the diagnosis of bleeding disorders (e.g. congenital hemophilia, VWD)
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5.14 Apply an understanding of the function, limitations and applications of global tests for bleeding states (e.g. thromboelastography/-metry, thrombin generation)
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