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Yuan SR, Cao L, Li XJ, Cao Y. Acquired hemophilia A following choledochal cyst excision - highlighting postoperative bile leak and infection as potential triggers: A case report. World J Gastrointest Surg 2026; 18(11): 125182 [DOI: 10.4240/wjgs.125182]
World J Gastrointest Surg. Nov 27, 2026; 18(11): 125182 Published online Nov 27, 2026. doi: 10.4240/wjgs.125182
Acquired hemophilia A following choledochal cyst excision - highlighting postoperative bile leak and infection as potential triggers: A case report
Shun-Rong Yuan, Li Cao, Xiao-Ju Li, Yong Cao
Shun-Rong Yuan, Li Cao, Yong Cao, Department of Hepatobiliary Surgery, Southwest Hospital, Army Medical University, Chongqing 400038, China
Xiao-Ju Li, Department of Hepatobiliary Surgery, Qujing Second People’s Hospital of Yunnan Province, Qujing 655000, Yunnan Province, China
Co-corresponding authors: Xiao-Ju Li and Yong Cao.
Author contributions: Yuan SR collected the clinical data and prepared the figures, drafted the manuscript; Cao Y and Li XJ conceived and designed the study; Cao L and Cao Y critically revised the manuscript and approved the final version; and all authors read and approved the final manuscript. Both Cao Y and Li XJ have played important and indispensable roles in the manuscript design, data interpretation and manuscript preparation as the co-corresponding authors. Li XJ applied for and obtained the funds for this research project. Cao Y conceptualized, designed, and supervised the whole process of the project. He searched the literature, revised and submitted the early version of the manuscript with the focus on the postoperative bile leak and infection as potential triggers.
AI contribution statement: No use of AI tools.
Supported by the Special Funding for the Cultivation of High-level Health Technology Professionals in Yunnan Province, No. H-2025079.
Informed consent statement: Written informed consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: All authors declare that they have no conflict of interest to disclose.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Corresponding author: Yong Cao, MD, Department of Hepatobiliary Surgery, Southwest Hospital, Army Medical University, No. 30 Gaotanyan Street, Shapingba District, Chongqing 400038, China. caoyong821@tmmu.edu.cn
Received: July 6, 2026 Revised: August 12, 2026 Accepted: September 20, 2026 Published online: November 27, 2026 Processing time: 134 Days and 22.5 Hours
Abstract
BACKGROUND
Acquired hemophilia A (AHA) is a rare and potentially fatal bleeding disorder mediated by autoantibodies against coagulation factor VIII (FVIII). Its nonspecific clinical presentation often leads to delayed diagnosis and life-threatening complications. Early recognition and standardized management are crucial for improving outcomes. Secondary AHA following choledochal cyst excision is extremely rare, with no prior reports in the literature. This case report explored the possible etiology, treatment strategies, and disease course of AHA in a patient.
CASE SUMMARY
A 37-year-old woman developed postoperative bile leakage, infection, and recurrent bleeding after laparoscopic choledochal cyst excision with Roux-en-Y hepaticojejunostomy, which was subsequently complicated by AHA. On postoperative day 6, she presented with intra-abdominal hemorrhage. Despite repeated interventions, including exploratory laparotomy and interventional embolization, intermittent active bleeding remained uncontrolled. After multidisciplinary team consultation, she was finally diagnosed with AHA. Treatment included infection control, combined immunosuppressive therapy with corticosteroids and cyclophosphamide, seven plasma exchange sessions, and FVIII replacement therapy. Bleeding was effectively controlled, the intra-abdominal abscess healed, and the patient was discharged after clinical remission was achieved. At the 6-month follow-up, no signs of recurrent bleeding were noted.
CONCLUSION
Prompt FVIII activity and inhibitor testing, along with comprehensive treatment, should be implemented in patients suspicious for AHA.
Core Tip: This report describes the first case of acquired hemophilia A after cholangiocystectomy. Postoperative bile leak with persistent abdominal infection is the key trigger for anti-factor VIII (FVIII) antibody formation. Timely FVIII testing, infection control, immunosuppression, and plasma exchange achieve cure.
