タイトル(掲載誌)International journal of hematology
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 clinical
severity and risk of inhibitor development in patients with severe hemophilia B (Pw-SHB). However, such measurement
sensitivity 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-SHB
plasmas, 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 compared
to its absence. The addition of rFIX to these innate plasma samples produced significant changes in three parameters
upon adding FIX:C at 0.1–1 IU/dL, supporting the presence of trace FIX:C in Pw-SHB. Therefore, appropriate FIX-depleted
plasma containing minimum residual FIX:C was chosen from reference curves of FIX:C (0.01–1 IU/dL). Among patients
with 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 very
low 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
関連情報(DOI)10.1007/s12185-022-03419-4
連携機関・データベース国立情報学研究所 : 学術機関リポジトリデータベース(IRDB)(機関リポジトリ)
提供元機関・データベース奈良県立医科大学 : 奈良県立医科大学機関リポジトリ GINMU