Recommendations on Acute Bleed Management – PwHA without inhibitors
No serious adverse events have been observed related to concomitant use of FVIII concentrates in patients on emicizumab prophylaxis. Specifically, no thrombotic or TMA events have been observed.
Emicizumab is likely to transform the bleeding phenotype of PwHA without inhibitors to a milder phenotype with a significant reduction in bleeding events requiring treatment. Significant and serious or life-threatening bleeding should continue to be treated promptly.
• All FVIII concentrates (plasma-derived and recombinant standard half-life and extended half-life) may be used for breakthrough bleeding events. Dosing should follow the same recommendations as when the patient was on FVIII replacement therapy .
• Antifibrinolytics may be used in combination with FVIII concentrates when clinically indicated for mucosal bleeding or for minor oral procedures.
Recommendations on surgical management with emicizumab..
Emicizumab is approved for prophylaxis, but how this extends to surgical prophylaxis remains to be fully understood.
While it improves hemostasis, it does not normalize hemostasis. This is especially important when planning hemostatic control in the surgical setting. Within the clinical trials, some patients had adequate hemostatic control with emicizumab alone for minor surgical procedures while others did not.
This is like what has historically been seen in patients with mild hemophilia.
a. Surgeries should be conducted at centers with appropriate experience and access to necessary laboratory monitoring assays for concomitant use of hemostatic agents.
b. Elective surgeries should be conducted after PwHA have completed the loading dose phase and are at steady state maintenance dosing (following week 5).
c. Emicizumab alone should not be presumed to be adequate for major procedures where current standards of care are to maintain factor levels within the normal range for a period of days.
d• Close monitoring of bleeding control as well as access to appropriate laboratory assays to monitor therapy (eg. chromogenic FVIII assay with FVIII replacement) is very important when determining treatment plans for patients on emicizumab needing surgical procedures.
e. For major surgeries and procedures where bleeding could result in serious complications, patients should be provided rFVIIa or FVIII replacement pre- and post-operatively to maintain adequate hemostasis at the discretion of the treating physician. Anti-fibrinolytics may also be part of the perioperative management plan. Further research/experience is needed to form better defined treatment plans for different surgical procedures.
f. Providers are cautioned to consider that bleeding complications from surgeries in patients with hemophilia still greatly outweigh thrombotic complications in frequency and morbidity/mortality.
reference MASAC document
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