Clinical Practice and Cases in Emergency Medicine

Introduction: Hemophilia A is an X-linked recessive bleeding disorder characterized by deficiency of factor VIII. The clinical features of this disease are hemorrhage and can be life-threatening when major organ systems are involved.

Case Report: We report the case of a 13-year-old boy with severe hemophilia A and factor VIII inhibitors who presented with fever and septic arthritis, later developing refractory shock with tachyarrhythmia. Despite initial suspicion of sepsis-related myocarditis, point-of-care ultrasound (POCUS) revealed massive hemorrhagic pericardial effusion with tamponade physiology. Recombinant factor VIIa was transfused as hemostatic rescue therapy; the patient was subsequently started on fresh frozen plasma and emicizumab. Ultrasound-guided pericardiocentesis resulted in rapid hemodynamic recovery.

Conclusion: This case highlights the importance of considering obstructive shock in unstable hemophilia patients and demonstrates that timely POCUS-based diagnosis and factor-supported pericardiocentesis can be lifesaving.