一般注記type:Thesis
The precise measurement of very low levels of factor IX activity (FIX:C < 1 IU/dL) is essential for understanding clinicalseverity and risk of inhibitor development in patients with severe hemophilia B (Pw-SHB). However, such measurementsensitivity has not yet been achieved. We aimed to establish a measurement method using clot waveform analysis (CWA).Residual FIX:C by adding anti-FIX monoclonal antibody, FIX:C by adding recombinant (r)FIX to the commercial Pw-SHBplasmas, and FIX:C in our Pw-SHB were determined by CS-2000i™/CS-2400™, followed by analysis of CWA parameters.The presence of anti-FIX antibody in the commercial Pw-SHB plasmas significantly decreased coagulation potential comparedto its absence. The addition of rFIX to these innate plasma samples produced significant changes in three parametersupon adding FIX:C at 0.1–1 IU/dL, supporting the presence of trace FIX:C in Pw-SHB. Therefore, appropriate FIX-depletedplasma containing minimum residual FIX:C was chosen from reference curves of FIX:C (0.01–1 IU/dL). Among patientswith untreated Pw-SHB, two had FIX:C 0.6–0.7 IU/dL and two had lower than detectable levels using FIX-depleted plasma.One of the latter had detectable trough levels post-rFIX administration. In conclusion, CWA enabled measurement of verylow levels of FIX:C using appropriate FIX-deficient plasma.
博士（医学）・乙第1528号・令和4年12月22日
identifier:International journal of hematology,Vol.116 No.5 p.778-786 (2022)
identifier:09255710
identifier:http://ginmu.naramed-u.ac.jp/dspace/handle/10564/4094
identifier:International journal of hematology, 116(5): 778-786
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