Sequential therapy with bypassing agents is sometimes used for hemostatic management in hemophilia patients with inhibitors, but there is no established therapy. In this study, we evaluated the coagulation capacity of a hemophilia B patient with inhibitors treated with factor VIIa/factor X (FVIIa/FX) and high-dose recombinant FVIIa (rFVIIa) sequential therapy. The patient, a teenage male, was treated with these agents in the order of FVIIa/FX and high-dose rFVIIa during major bleeding that was difficult to stop. The activated partial thromboplastin time (APTT) was shortened and thrombin generation and FVII antigen levels were increased after treatment compared to before treatment. On the other hand, FX antigen level was increased only after FVIIa/FX administration. Compared to high-dose rFVIIa single administration, the coagulation capacity after additional high-dose rFVIIa administration on the day after FVIIa/FX administration tended to be stronger. No signs of overcoagulation were observed with the sequential therapy. The results suggest that additional rFVIIa administration may be a reasonable hemostatic strategy for refractory hemorrhage in hemophilia B patients with inhibitors when plasma FX levels are maintained at high levels after FVIIa/FX administration.

Background: To understand barriers of physical activity in people with hemophilia is considered to be a key for improving quality of life in long-term, yet there are no linguistically validated questionnaires to assess the patient-reported barriers in Japanese patients with hemophilia.

Object: We aim to linguistically validate the original English version of the Barriers to Being Active Quiz (BBAQ-21) into Japanese version for its future usage in Japanese hemophilia patients.

Method and Results: One forward translated version of BBAQ-21 was created from two independent translations, then back translation was performed from the forward translation and confirmed conceptual equivalency with the original English version. Cognitive interviews to patients with hemophilia were conducted to confirm the linguistic validity of the forward translated version and to identify points that needed further correction, and the Japanese version of BBAQ-21 was finalized accordingly.

Conclusion: The Japanese version of BBAQ-21 was created, and its linguistic validity was confirmed through cognitive interview. Through the usage of linguistically validated version of Japanese BBAQ-21 questionnaire, it will allow researchers to investigate the potential barriers that associate with physical activity among Japanese patients with hemophilia.