Surgery-associated acquired hemophilia A: a report of 2 cases and review of literature
- PDF / 297,729 Bytes
- 7 Pages / 595.276 x 790.866 pts Page_size
- 37 Downloads / 166 Views
CASE REPORT
Open Access
Surgery-associated acquired hemophilia A: a report of 2 cases and review of literature Umar Zeb Khan1, Xiangwu Yang1, Matiullah Masroor2, Abdul Aziz3, Hui Yi3 and Hai Liu1*
Abstract Background: Acquired Hemophilia A (AHA) is a rare bleeding diathesis in patients with no previous personal or family bleeding history. The diagnosis of this disease often delays due to unfamiliarity of physicians with it, which leads to its high mortality rate. Case presentation: Two cases (one 12 years old female and another 18 years old male) were admitted for right upper abdominal mass and right upper abdominal pain respectively at different times. Pre-operative diagnosis of both cases was congenital choledochal cyst. They suffered continuous gastrointestinal bleeding (hematemesis and melena) with reduced hemoglobin to 54 g/L and 60 g/L after Roux-en-Y anastomosis respectively. To investigate the exact bleeding site, Digital subtraction angiography (DSA) of case 1 showed contrast overflow at small branch of proper hepatic artery but had unremarkable result for case 2, whereas gastroscopy of both cases showed unremarkable results. Multiple surgeries were also performed for hemostatic purpose but each time no active bleeding site was found. Finally, hematologists consultation was mandated in both cases and they were diagnosed as acquired haemophilia A. However, unfortunately case 1 patient could not survive because of sever hemorrhage and infection while Case 2 of 18 years old male survived after proper haemophilia treatment catalog. Conclusion: Awareness about surgery associated acquired haemophilia A (SAHA) can facilitate quick diagnosis and lifesaving management because the mortality rate in SAHA is high due to lake of knowledge or late recognition of the disease. Bleeding always occurs at surgical sites and it can occur immediately within few hours after surgery in some cases. Hemorrhage may be severe or even life threatening and it presents a special challenge for diagnosis and treatment in a patient who has just undergone a surgical procedure. The treatment strategies for AHA include resumption of hemostasis with either recombinant porcine factor VIII (rpFVIII) or bypassing agents and immunosuppressive therapy to suppress the production of the factor VIII inhibitor. Keywords: Acquired hemophilia A, Acquired hemophilia surgeries, Factor VIII inhibitors, SAHA
Background Acquired hemophilia A (AHA) is an ultra-rare bleeding disorder, about 1.48 incidence per million occurs per year, characterized by reduction of factor VIII (FVIII) activity due to the development of autoantibodies against FVIII [1–3]. These autoantibodies breakdown and neutralize the hemostatic function of factor VIII which * Correspondence: [email protected] 1 Department of General Surgery, The Third Xiangya Hospital of Central South University, 138 Tongzipo Rd, Changsha 410013, China Full list of author information is available at the end of the article
leads to AHA in patients having no previous personal or family bleeding history [
Data Loading...
