Autoimmune Acquired Factor V Deficiency in a Patient With Pancreatic Cancer Complicated by Cholangitis: A Rare Coagulopathy With a Favorable Course

TL;DR

A rare bleeding disorder called autoimmune acquired factor V deficiency was diagnosed in a patient with pancreatic cancer and cholangitis, and it improved after treating the cholangitis.

Contribution

This case report highlights AiFVD associated with cholangitis due to biliary obstruction in pancreatic cancer, offering insight into a rare coagulopathy.

Findings

AiFVD was diagnosed in a 78-year-old woman with pancreatic cancer and cholangitis.

The coagulopathy resolved after treating the underlying cholangitis.

The case suggests a link between biliary obstruction and AiFVD.

Abstract

Autoimmune acquired factor V deficiency (AiFVD) is a rare bleeding disorder characterized by the presence of inhibitors against coagulation factor V (factor V). It can manifest with a wide spectrum of clinical severity, from asymptomatic to life-threatening bleeding. Various underlying causes have been reported, including malignancies, autoimmune disorders, infections, and antibiotics. However, the mechanism remains unclear, and diagnostic delays are common due to its rarity. A 78-year-old woman with pancreatic head cancer underwent bile duct stenting for malignant biliary stricture. She subsequently developed cholangitis from stent obstruction, accompanied by coagulopathy. Coagulation studies revealed a prolonged prothrombin time of 68.1 seconds, an activated partial thromboplastin time exceeding 283.7 seconds, and severely reduced FV activity (<3%). A cross-mixing test suggested the presence of an inhibitor, leading to the diagnosis of AiFVD. The coagulopathy resolved following treatment of the underlying cholangitis. AiFVD can arise from various causes; in this case, it was attributed to cholangitis caused by recurrent biliary obstruction.

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