علاج الهيموفيليا ب الشديدة أو متوسطة الشدة مع نشاط العامل التاسع أقل من 2 وحدة دولية/ديسيلتر وبدون مثبطات

References

1. In individuals with severe and moderately severe hemophilia B without inhibitors, the ISTH Hemophilia Guideline Panel recommends prophylaxis over episodic treatment of bleeding events (strong recommendation, based on moderate-certainty evidence ⨁⨁⨁◯).
2. Therefore, we defined hemophilia as severe and moderately severe when individuals enrolled in the study population had FVIII or FIX activity levels <2 IU/dL.
3. In individuals with severe and moderately severe hemophilia B without inhibitors, the ISTH Hemophilia Guideline Panel suggests prophylaxis with purified plasma-derived FIX or standard or extended half-life recombinant FIX concentrates (conditional recommendation, based on very low–certainty evidence ⨁◯◯◯).
4. In this guideline, standard prophylaxis refers to dose and dose intervals of 20 to 60 IU/kg of FIX twice per week for hemophilia B as defined by Srivastava et al.
5. The prophylactic doses, brand, and frequency of FIX infusion varied between the trials: nonacog beta pegol 10 to 40 IU/kg once weekly, albutrepenonacog alfa 35 to 50 IU/kg once weekly or 75 IU/kg every 10 or 14 days, and eftrenonacog alfa 50 IU/kg once weekly or 100 IU/kg every 10 days.