Repeated hemorrhagic ulcers of the esophagus associated with acquired hemophilia A: A case report.

backgroundAcquired hemophilia A (AHA) is a rare blood disorder that can cause life-threatening severe bleeding because of the development of autoantibodies (inhibitors) against factor VIII. AHA is common in elderly patients, and although most cases are characterized by subcutaneous or intramuscular bleeding, rare cases of gastrointestinal bleeding have been reported. Controlling gastrointestinal bleeding in patients with AHA is difficult even after multiple endoscopic hemostasis or transcatheter arterial embolization procedures, and these patients are prone to rebleeding. CASE SUMMARY: We report the case of a 76-year-old Japanese woman with AHA who presented with repeated bleeding from an esophageal ulcer as the initial symptom. A hemorrhagic ulcer was detected in the lower esophagus, and endoscopic hemostasis using radiofrequency ablation was performed seven times over a month. However, this procedure was unsuccessful, and the patient was transferred to our department. Hypertonic saline - epinephrine injection plus clipping was administered for endoscopic hemostasis, but the treatment response of the patient was poor. After the AHA diagnosis was confirmed, a single dose of a mixture of factors VIIa and X was administered as a bypass hemostatic agent, and the bleeding was successfully controlled. In addition, emicizumab, a bispecific monoclonal antibody that is a substitute for activated factor VIII, was administered, and no further rebleeding was observed.

conclusionMultidisciplinary interventions between endoscopists and hematologists are essential to manage rare gastrointestinal bleeding caused by coagulation disorders, such as AHA.