Emergency management in patients with haemophilia A and inhibitors on prophylaxis with emicizumab: AICE practical guidance in collaboration with SIBioC, SIMEU, SIMEUP, SIPMeL and SISET

Abstract

Emicizumab has been approved in several countries for regular prophylaxis in patients with congenital haemophilia A and FVIII inhibitors because it substantially reduces their bleeding risk and improves quality of life. However, although significantly less frequent, some breakthrough bleeds may still occur while on emicizumab, requiring treatment with bypassing or other haemostatic agents. Thrombotic complications have been reported with the associated use of activated prothrombin complex concentrates. In addition, when surgery/invasive procedures are needed while on emicizumab, their management requires multidisciplinary competences and direct supervision by experts in the use of this agent. Given this, and in order to expand the current knowledge on the use of emicizumab and concomitant haemostatic agents, and reduce the risk of complications in this setting, the Italian Association of Haemophilia Centres (AICE) here provides guidance on the management of breakthrough bleeds and surgery in emergency situations in patients with haemophilia A and inhibitors on emicizumab prophylaxis. This paper has been shared with other National Scientific Societies involved in the field.

Figure 1

Algorithm for management of emergency situations in patients with haemophilia A and inhibitors on emicizumab prophylaxis It is recommended that the HTC be consulted to evaluate and confirm treatment with bypassing agents, to define clinical outcome and, thereafter, to determine duration of treatment. In case of no/unsatisfactory response to the first-line treatment, the evaluation and management of second-line therapy approaches, as described in this document, should be made under the direct control and responsibility of the HTC. In cases in which an institutional HTC on-call is not available, the referral protocols will be granted and agreed between the HTC and the Emergency Unit, and formally approved by the hospital management. *If necessary to clarify the diagnosis and to decide indication to treatment. HTC: Hemophilia Treatment Centres; rFVIIa: recombinant activated FVII.