Recurrent Postoperative Hemorrhage Unmasks Mild Hemophilia A in a Cholangiocarcinoma Patient

Introduction: Postoperative hemorrhage can result from surgical bleeding, infection, or underlying coagulopathy. Obstructive cholestasis predisposes to vitamin K malabsorption and acquired coagulopathy. Mild hemophilia A often remains undiagnosed until major surgery or trauma triggers excessive bleeding. We present a case of refractory post-hepatectomy bleeding unmasking vitamin K deficiency and previously unrecognized mild hemophilia A.


Description: A 32-year-old man with hilar cholangiocarcinoma underwent extended hepatectomy with Roux-en-Y hepaticojejunostomy. His postoperative course was complicated by four distinct episodes of major intra-abdominal bleeding confirmed by computed tomographic angiography. He required three separate ICU admissions for hemorrhagic shock. He underwent two exploratory laparotomies for washout and hematoma evacuation, and two interventional radiology procedures for empiric embolization of pseudoaneurysms identified in branches of the mesenteric artery and right hepatic artery. Hematology was consulted, given recurrent bleeding in the absence of a surgical source. Coagulation studies revealed prolonged PT/INR and aPTT. Factor assays showed reduced levels of vitamin K–dependent factors (FVIII 37%, FVII 43%, FII 42%), with vWF antigen >390%. Notably, factor VIII was low-normal (37%) - inappropriately low in this inflammatory, post-operative state where levels should be elevated due to its role as an acute phase reactant. These findings raised concern for vitamin K deficiency and previously undiagnosed hemophilia A. He was treated with vitamin K, plasma, and recombinant factor VIII, with eventual bleeding resolution.


Discussion: This case highlights the importance of evaluating for both acquired and congenital coagulopathies in unexplained postoperative bleeding. The combination of vitamin K deficiency and unrecognized mild hemophilia A led to recurrent hemorrhage despite appropriate surgical and interventional care. The inappropriately low factor VIII level, despite elevated vWF and systemic inflammation, was key to diagnosing mild hemophilia A. Targeted therapy including factor VIII replacement was essential for bleeding control, emphasizing the need for comprehensive hemostatic evaluation in complex surgical ICU patients.