Yes or no for secondary prophylaxis in afibrinogenemia?

3D Soft-Tissue Prediction Methodologies for Orthognathic Surgery—A Literature Review

“…Moreover, surgeons must manage patients with rare disorders of hemostasis, which need particular care in the perioperative phase. The works by Simurda et al [38,39], and Ghadimi et al [40] have been referred to in order to deepen understanding of this topic.…”

Perioperative Coagulation Management in a Patient with Congenital Afibrinogenemia during Revision Total Hip Arthroplasty

“…3,4 The estimated prevalence of afibrinogenemia is around 1 in 1,000,000, 4 although in Slovakia we estimate it to be 1 in 5,000,000. 5 The commonest manifestation of the disease is bleeding from mucosal surfaces; however, musculoskeletal bleeding, gynecologic and obstetric complications, spontaneous bleeding, bleeding after minor trauma and during interventional procedures, or thromboembolic episodes have also been reported. 6 Absence of immunoreactive fibrinogen is essential for the diagnosis of congenital afibrinogenemia.…”

“…This patient was managed with prophylactic intravenous administration of fibrinogen concentrate at the dose of 2 g once a month from the age of 15 years. 5 Since then, he had significant reduction in the intensity and frequency of spontaneous bleeding.…”

Severe female hemophilia A patient caused by a nonsense mutation (p.Gln1686X) of F8 gene combined with skewed X-chromosome inactivation

“…We decided for such regimen according to the information about successful avoidance of bleeding and improvement in quality of life after the prophylactic management with the administration of replacement clotting factor when compared with on-demand regimen in haemophiliacs [7]. Since then, he noticed significant reduction of the frequency and intensity of spontaneous bleeding.…”