Figure 1. Improvement in HJHS recorded 6 monthly. HJHS: hemophilia joint health score.
| Journal of Hematology, ISSN 1927-1212 print, 1927-1220 online, Open Access |
| Article copyright, the authors; Journal compilation copyright, J Hematol and Elmer Press Inc |
| Journal website https://www.thejh.org |
Original Article
Volume 13, Number 5, October 2024, pages 186-191
Freedom From Bleeds With Low-Dose Emicizumab Prophylaxis in Inhibitor-Positive Hemophilia A
Figures
Tables
| N | Standard dose (N = 7) | Low dose (N = 25) |
|---|---|---|
| HJHS: hemophilia joint health score; ABR: annualized bleeding rate. | ||
| Median age in years (range) | 8 (5 - 11) | 12 (3 - 28) |
| Median duration of follow-up in months (range) till September 2023 | 27 (24.5 - 27.3) | 13.5 (7.7 - 24) |
| Median baseline HJHS | 9 | 12 |
| Median post treatment HJHS at 6 months of prophylaxis (P = 0.69) | 6 | 6 |
| Median post treatment HJHS at 12 months of prophylaxis (P = 0.89) | 0 | 4 |
| Baseline ABR (median) | 15 | 16 |
| Treated bleeds within 6 months of starting emicizumab | 0 | 1 |
| Median post-treatment ABR at 12 months of prophylaxis (P = 0.96) | 0 | 0 (1 treated bleed) |
| Year 1 (cost/ number of doses) | Year 2 onwards (cost/ number of doses) | |
|---|---|---|
| HAI: hemophilia A patients with inhibitors; INR: Indian rupees. | ||
| Standard-dose emicizumab | $4,628 | $4,269 |
| INR 379,680 | INR 352,560 | |
| For 28 doses | For 26 doses | |
| Low-dose emicizumab | $2,134 | $2,134 |
| INR 203,400 | INR 203,400 | |
| For 13 doses | For 13 doses | |
| Average dose used for one bleed for 10-kg child | Cost per bleed | |
|---|---|---|
| rFVIIa: recombinant factor VIIa; aPCC: activated prothrombin complex concentrates; INR: Indian rupees. | ||
| aPCC | 50 IU/kg/dose twice a day, for 4 days | INR 208,000 |
| Modeled at cost of INR 26,000 per 500 IU vial | ||
| rFVIIa | 1 mg thrice a day for 2 days | INR 264,000 |
| Modeled at cost of INR 44,000 per 1 mg vial | ||