Two pediatric cases of severe hemophilia A in which emicizumab prophylaxis failed to prevent traumatic extra-articular hemorrhage.

Abstract

Emicizumab reduces bleeding episodes in patients with severe hemophilia A (PwHA). Little information is available on hemostatic management of severe traumatic hemorrhages in emicizumab-treated pediatric PwHA. We assessed therapeutic efficacy and global coagulation potentials in two pediatric cases of emicizumab-treated pediatric PwHA with intracranial or retroperitoneal/iliopsoas hemorrhage. A modified clot waveform analysis (CWA) triggered by mixtures of tissue factor and ellagic acid was used to assess coagulant potentials, and maximum coagulant velocity (Ad|min1|) was calculated. One patient with intracranial hemorrhage was treated with continuous infusions of recombinant factor VIII (rFVIII) at a dose of 4-4.6 IU/kg/hr for 9 days, followed by bolus infusion at 66 IU/kg/day for 2 days and 33 IU/kg/day for an additional 2 days. The Ad|min1| was increased from 5.5 (at baseline) to 7.0-8.1 under concomitant treatment and maintained within or near normal range (IQR; 6.9-7.7). The other patient with retroperitoneal/iliopsoas hemorrhage received bolus infusions of rFVIII at 50 IU/kg/day for 20 days and every-other-day infusion of rFVIII for 8 days. The Ad|min1| was increased from 5.2 (at baseline) to 5.8-6.8 under concomitant treatment and maintained within the normal range. We successfully managed a treatment plan for severe traumatic bleeding in emicizumab-treated pediatric PwHA using modified CWA.

• Authors: Yuya Yamada, Yuto Nakajima, Ayaka Ohara, Emi Wakita, Kazuki Shimizu, Naruto Shimonishi, Shoko Furukawa, Kenichi Ogiwara, Masahiro Takeyama, Keiji Nogami
• Journal: International journal of hematology (Int J Hematol) Vol. 117 Issue 4 Pg. 607-612 (Apr 2023) ISSN: 1865-3774 [Electronic] Japan
• PMID: 36370317 (Publication Type: Journal Article)
• Copyright: © 2022. Japanese Society of Hematology.

Chemical References

• emicizumab
• Factor VIII
• Antibodies, Bispecific

Topics

• Humans
• Child
• Hemophilia A (complications, drug therapy)
• Hemorrhage (etiology, prevention & control)
• Factor VIII (therapeutic use)
• Antibodies, Bispecific (therapeutic use)