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Korean Journal of Otorhinolaryngology-Head and Neck Surgery > Volume 58(11); 2015 > Article
Korean Journal of Otorhinolaryngology-Head and Neck Surgery 2015;58(11): 781-785.
doi: https://doi.org/10.3342/kjorl-hns.2015.58.11.781
Unexpected Bleeding Following Endoscopic Sinus Surgery Leading to the Rare Diagnosis of Acquired Haemophilia A.
Sung Hwan Ahn, Yong Won Kim, Dong Hyuk Im, Jin Kook Kim
Department of Otorhinolaryngology-Head and Neck Surgery, Konkuk University School of Medicine, Seoul, Korea. entalk@kuh.ac.kr
내시경적 부비동 수술 이후 예기치 못한 출혈로 후천성 혈우병 A로 진단된 증례
안성환 · 김용운 · 임동혁 · 김진국
건국대학교 의학전문대학원 이비인후-두경부외과학교실
We report a case of acquired hemophilia A following endoscopic sinus surgery. The patient was a 64-year-old man whose preoperative activated partial thromboplastin time (aPTT) was slightly prolonged. He underwent endoscopic sinus surgery for chronic rhinosinusitis with nasal polyp. Nasal bleeding occurred spontaneously on the postoperative day 3. The aPTT was prolonged at 58.9 s, with factor VIII activity of 21% and a positive factor VIII inhibitor. Treatment with factor VIII and methylprednisolone resulted in remission within 1 month. This is the first of such case reported in Korea; the patient was treated by recombinant factor VIII replacement and steroid therapy.
Keywords: Acquired hemophilia AEndoscopic sinus surgeryFactor VIIIFactor VIII inhibitorSteroid

Address for correspondence : Jin Kook Kim, MD, PhD, Department of Otorhinolaryngology-Head and Neck Surgery, Konkuk University School of Medicine, 120-1 Neungdong-ro, Gwangjin-gu, Seoul 05030, Korea
Tel : +82-2-2030-7662, Fax : +82-2-2030-5299, E-mail : entalk@kuh.ac.kr


Acquired hemophilia A (AHA) is an autoimmune disease caused by autoantibodies that inhibit the function of factor VIII (FVIII).1,2,3,4) The clinical characteristics of the disease are diverse ranging from superficial bleeding to life-threatening bleeding and it has an incidence of 1 in 1.48 million per year.5) Nowadays, endoscopic sinus surgery is most commonly performed for surgical treatment of chronic rhinosinusitis with nasal polyp.6,7) Complications associated with endoscopic sinus surgery are bleeding, cerebrospinal fluid rhinorrhea, adhesion, orbital complications, and nasolacrimal duct damage. Among the complications, bleeding is the most common complication, and if bleeding occurs during the surgery, it can interfere with the view of the surgical field, and increase the risk of other complications.8) For this reason, patients who use a thrombolytic agent such as aspirin or have blood clotting disorders should be carefully examined at the time of surgery. We report a case of a patient who was diagnosed with AHA due to unexpected persistent bleeding after endoscopic sinus surgery and was treated with recombinant FVIII, blood transfusions, steroids, etc.


A 64-year-old male patient was planning to undergo endoscopic sinus surgery under general anesthesia due to chronic sinusitis. The patient had no specific past medical history and had received hemorrhoidectomy 10 years ago without any complications.
Right frontal sinusitis and right and left ethmoidal sinusitis were confirmed by paranasal sinus CT (Fig. 1). Initial coagulation studies at our institution showed a prolonged activated partial thromboplastin time (aPTT) (54.0 sec) (reference range, 29-45 sec), and there were no other abnormal pre-operative laboratory findings. Re-examination was performed due to the prolonged aPTT, and the result still showed a prolonged aPTT (51.0 sec). We consulted the department of hemato-oncology regarding the patient's high risk of bleeding, and an additional study was performed. In the immediate 1:1 mixing study with normal plasma, the prolonged aPTT seemed to have corrected (32.6 sec). The mixing test results with pre-incubation and prolonged incubation for 2 hr at 37℃ showed further prolongation of the aPTT (41.6 and 47.8 sec, respectively), and the result of the retest showed a slightly prolonged in the aPTT (48.2 sec). Hence, we performed the surgery as planned. There were no complications including bleeding immediately after surgery; hence, the patient was discharged after packing out cotton in both nasal cavities on post-operative day 2.
At 3 days after the operation during the follow-up visit to the hospital for sinus irrigation, admission was recommended due to persistent mild bleeding. But, the patient strongly refused admission. On postoperative 10 days, The patient was re-admitted due to uncontrolled bleeding and endoscopic-assisted cauterization and packing was performed under local anesthesia in the operation room for achieving bleeding control. With respect to surgical findings, there was diffuse mucosal bleeding in the left nasal cavity and neither a clear bleeding focus nor exposed vessels were observed. Electric cauterization, application of Surgicel® (ETHICON Sarl, Neuchatel, Switzerland), and insertion of the packing materials such as Rapid Rhino® (Arthro Care, UK Ltd., Sunnyvale, CA, USA) were performed for achieving hemostasis (Fig. 2).
After the surgery, bilateral epistaxis was still persistent. Therefore, we re-consulted the department of hemato-oncology, and retest was performed. FVIII activity was decreased to 21% (reference range, 60-140%), aPTT was prolonged (58.9 sec), FVIII antibody (inhibitor) was negative. Therefore, fresh frozen plasma and red blood cell transfusion and cryoprecipitate transfusion, and recombinant FVIII replacement therapy were performed under suspicion of blood clotting factor (FVIII) deficiency. However, despite continuous treatment, persistent epistaxis and FVIII deficiency, and prolonged aPTT showed no changes; hence, we re-consulted the department of hemato-oncology. The result of FVIII antibody (inhibitor) test under suspicion of AHA was positive on the second attempt. Therefore, the patient was diagnosed with AHA and the use of a bypassing agent such as recombinant activated factor VII was considered. However, since recombinant activated factor VII is very expensive, FVIII replacement and high dose steroid therapy (methylprednisolone IV, 1 mg/kg) were added to the treatment after considering the socio-economic status of the patient.
Nasal bleeding was significantly reduced after the use of steroids, and at 5 days after starting the steroid therapy, FVIII activity was increased to 45%, and the aPTT was decreased to 49.5 sec compared with the initial findings (Fig. 3). The patient was discharged without nasal bleeding and the final diagnosis was AHA. No more nasal bleeding was observed after discharge (Fig. 4). At follow-up, these parameters were maintained in the normal range; FVIII activity was 123%, aPTT was 34.1 sec. The patient is being followed up at the department of hemato-oncology, internal medicine and otorhinolaryngology-head and neck surgery.


Although some cases have been reported that regional bleeding after endoscopic sinus surgery but case accompanied by systemic disease after endoscopic sinus surgery is not been reported.9,10) Other than the FVIII deficiency or inhibitor, there are rare bleeding disorders such as vitamin K deficiency, disseminated intravascular coagulation, von Willebrand disease, thrombocytopenia, liver failure, factor V, X, XII deficiency. AHA is a rare autoimmune disease that mainly presents as spontaneous bleeding (epistaxis, hematuria, postpartum hemorrhage, etc.).11,12) It may present as uncontrolled bleeding after trauma.
Although AHA is a very rare disease, it can occasionally be a life-threatening disease. Hence, early diagnosis and treatment would have a significant effect on prognosis of the patient. The diagnosis of AHA is made based on the correlation between clinical features and laboratory investigations. The most common laboratory abnormality is an isolated prolonged aPTT, with other coagulation factors in their normal range. The isolated prolonged aPTT may be due to deficiency of one of the intrinsic coagulation factors (FVIII, IX, XI, or XII) or it may suggest the presence of an inhibitor. It is also important to rule out nonspecific inhibitors (lupus anticoagulant or heparin) that could prolong the aPTT.11,13)
If a patient who has no prior history of bleeding tendency bleeds continuously in spite of normal result of blood tests except for a prolonged aPTT, we should suspect that he or she may have AHA. The most important points for confirmation of the diagnosis of AHA are a decrease in FVIII activity and the presence of FVIII antibody in the blood test.
If a physician suspects AHA clinically, he should first start the proper treatment because there can be false-negative results of FVIII antibody. The purpose of treating AHA is to prevent and treat the complications such as bleeding and to eradicate the FVIII antibody.3,14)
There are several treatment options for AHA. First option is to use bypassing agents such as recombinant activated factor VII and recombinant FVIII replacement therapy. We can also use corticosteroid mono-therapy or use corticosteroid and cyclophosphamide concurrently. An alternative option such as rituximab is also likely.13,14)
This review presents the first diagnosed case of AHA following endoscopic sinus surgery in Korea. Also, this is the first case to be treated with replacement of recombinant FVIII and steroid therapy although a bypassing agent such as recombinant activated factor VII is the first choice treatment agent.

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