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. 2023 May;117(5):669-677.
doi: 10.1007/s12185-022-03528-0. Epub 2023 Jan 6.

High levels of factor VIII activity in patients with acquired hemophilia A in remission are associated with unusually low coagulation potentials

Fumitaka Shibasaki  1 , Masahiro Takeyama  2 , Kenichi Ogiwara  3 , Shoko Furukawa  3 , Yuto Nakajima  3 , Naruto Shimonishi  3 , Keiji Nogami  3
Affiliations

High levels of factor VIII activity in patients with acquired hemophilia A in remission are associated with unusually low coagulation potentials

Fumitaka Shibasaki et al. Int J Hematol. 2023 May.

Abstract

Background: Elevated factor VIII activity (FVIII:C) is often observed in patients with acquired hemophilia A (PwAHA) in remission. However, comprehensive coagulation potentials in this patient group remain to be investigated.

Aim: To evaluate comprehensive coagulation potentials in PwAHA.

Methods: We investigated coagulation function in eleven PwAHA with high FVIII:C (> 150 IU/dL) using thrombin generation assay (TGA) and/or rotational thromboelastometry (ROTEM), and compared findings with results obtained from contrived samples generated by spiking recombinant FVIII.

Results: The median FVIII:C and FVIII inhibitor titers during remission in enrolled PwAHA were 206 IU/dL and 0.44 BU/mL, respectively. In all patients, lag time and time to peak were either prolonged or normal compared to contrived samples corresponding to their FVIII:C. However, higher values of peak thrombin and endogenous thrombin potentials compared to contrived samples were observed in two patients. ROTEM parameters were within normal ranges in all cases. One patient (FVIII:C 171 IU/dL) developed venous thrombosis and pulmonary embolism, but TGA parameters showed low or normal coagulation potential compared to contrived samples corresponding to his FVIII:C.

Conclusion: PwAHA with high FVIII:C could exhibit lower coagulation potentials than those corresponding to their FVIII:C.

Keywords: Acquired hemophilia A; Anti-factor VIII antibody; Blood coagulation test; Factor VIII; Factor VIII activity.

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