Abstract: Objective This study aims to examine the clinical features and methods of laboratory examination for the diagnosis of a rare hemophilia refractory epistaxis. Methods We reviewed and analyzed the patient's case data, summarized the characteristics of the case, and noted the deficiencies in the treatment process. Local and international literatures on hemophiliac rhinorrhea were also reviewed in the domestic and overseas. Results The patient presented with epistaxis without apparent cause as the first symptom, and have no space occupying lesions of the nasal cavity and nasopharynx. Initially, the patient underwent electrocoagulation treatment in the outpatient department, but the symptoms worsened immediately. After admission, the symptoms were still not relieved after undergoing anterior and posterior nasal tamponade and transfusion of coagulation factors. Only a few hours after the operation, the condition worsened again. After nasal plasma electrocoagulation under general anesthesia, the bleeding was successfully stopped and has not recurred thus far. Conclusion Hemophilia is a type of congenital hemorrhagic disease with X-linked recessive inheritance, and a nosebleed is one of its main symptoms. The possibility of hemophilia should be taken into account in patients who have nosebleeds that do not have an obvious cause and persist after treatment. After hemophilia develops into refractory nosebleed, it is necessary to supplement the coagulation factors in time due before they are depleted. During interventional therapy, such patients may have received nasal tamponade or other treatments before surgery. Although the success rate of hemostasis after interventional surgery is relatively high, nasal endoscopy should be performed again after postoperative hemostasis. The suspected bleeding site is cauterized to stop the bleeding and avoid postoperative secondary bleeding. At the same time, a multidisciplinary team must be formed to identify the cause of bleeding and improve the clinical efficacy when the specialized treatment fails.

Key words: Hemophilia A, Refractory epistaxis, Nasal endoscopy, Angiography, Blood transfusion