Blood: Emicizumab treats obtained haemophilia A.

Obtained haemophilia A (AHA) is a serious hemorrhagic disease caused by inhibitory autoantibodies of the coagulation factor VIII (FVIII).
bypass therapy, human or pig FVIII is currently the standard treatment for AHA hemolysis.
Emicizumab is a dual-specific, FVIII-like therapeutic antibody that reduces the annual bleeding rate in patients with congenital haemophilia.
report, the researchers reported on the condition and efficacy of 12 AHA patients treated with emicizumab (6 men, 6 women, median age 74).
the initial activity of FVIII in all patients was less than 1% and the medium dose of inhibitors was 22.3BU/mL.
8 patients had severe bleeding.
Emicizumab starts at 3 mg/kg (sc), 2-3 times/week, and then changes to 1.5 mg/kg/3 weeks to maintain the lowest effective FVIII activity level.
used color assays of human and bovine reagents for FVIII testing.
all patients also received immunosuppression of steroids and/or lycoxi monotherapy.
the first dose of emicizumab, APTT returned to normal within 1-3 days, and the activity of FVIII increased to more than 10% after 11 days (medium time, range 7.5-12 days).
hemolysis after 1.5 days (1-4 days) and bypass therapy can be deactivated.
after the 31st day (15-79 days), the emicizumab was deactivation, with a median injection of 5 times (range 3-9), and after 115 days (67-185 days), FVIII (bovine reagent) activity exceeded 50%, suggesting complete remission.
no patients died of bleeding or thrombosis, and there was no sudden bleeding after the first emicizumab.
to sum up, emicizumab appears to be an effective hemolysis therapy for AHA, with the advantages of sc therapy, good hemolysis, shorter hospital stays, reduced immunosuppression and fewer adverse events.
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