[Surgery in patients with severe or inhibitory hemophilia A under prevention injection of emicizumab]. / Khirurgicheskie vmeshatel'stva u bol'nykh tyazheloi ili ingibitornoi formoi gemofilii A na fone profilakticheskogo vvedeniya emitsizumaba.

ABSTRACT

OBJECTIVE: To determine the features of preoperative hemostatic therapy and laboratory control in patients with severe and inhibitory forms of hemophilia A under preventive treatment by FVIII. MATERIAL AND METHODS: Four patients with severe and inhibitory forms of hemophilia A underwent surgery between 2021 and 2022. All patients received Emicizumab (the first monoclonal drug for «non-factor¼ therapy of hemophilia) for prevention of specific hemorrhagic signs of hemophilia. RESULTS: Surgical intervention under preventive Emicizumab therapy was essential. Additional hemostatic therapy was not carried out or performed in reduced mode. There were no hemorrhagic, thrombotic or other complications. Thus, the so-called «non-factor¼ therapy is one of the variants for uncontrollable hemostasis in patients with severe and inhibitory forms of hemophilia. CONCLUSION: Preventive injection of Emicizumab ensures certain buffer of hemostasis system and stable lower limit of coagulation potential. This is the result of stable concentration of Emicizumab when used in any of the registered forms regardless of age and other individual characteristics. The risk of acute severe hemorrhage is excluded, while the probability of thrombosis is not increased. Indeed, FVIII has higher affinity than Emicizumab and displaces Emicizumab from coagulation cascade that does not result summation of total coagulation potential.