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Background <p>Acquired factor V deficiency (AFVD) is a rare hemorrhagic disorder caused by autoantibodies against coagulation factor V. Clinical manifestations vary widely, ranging from asymptomatic cases to severe hemorrhage or even thrombosis, and diagnosis is often challenging due to its rarity and nonspecific presentation. Reported triggers include bovine thrombin, antibiotics, surgery, autoimmune diseases, infections, and malignancy, although up to 20% of cases are idiopathic. We report a case of AFVD in an elderly hemodialysis patient, in whom corticosteroid therapy was effective but ultimately complicated by fatal gastrointestinal bleeding.</p> Case presentation <p>An 87-year-old woman on hemodialysis for diabetic nephropathy was admitted with acute cholecystitis and treated with ceftriaxone. On day 16, she developed bleeding from a gastrostomy site with markedly prolonged prothrombin time (69.3&#xa0;s) and activated partial thromboplastin time (260.4&#xa0;s). Cross-mixing studies showed inhibitor patterns, and factor V activity was reduced to 1.3%, with an inhibitor titer of 33.3 Bethesda units/mL, confirming AFVD. Despite no bleeding from vascular access sites, she developed progressive anemia and gingival bleeding. Intravenous prednisolone (40&#xa0;mg/day) was initiated, resulting in normalization of coagulation parameters and recovery of factor V activity to 111.7% within 30 days. However, on day 57, she developed massive melena. Endoscopy revealed hemorrhagic duodenal ulcers with exposed vessels, requiring endoscopic hemostasis. Recurrent gastrointestinal bleeding occurred on day 61, and she died of hemorrhagic shock.</p> Conclusions <p>This case illustrates the diagnostic process and therapeutic challenges of AFVD in a hemodialysis patient. Corticosteroid therapy was effective in eradicating the inhibitor and restoring factor V activity, but fatal gastrointestinal bleeding occurred as a severe treatment-related complication. Clinicians should be aware of AFVD as a rare but important cause of acquired coagulopathy, and careful monitoring for corticosteroid-associated adverse effects is crucial, especially in elderly and critically ill individuals.</p>

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Acquired factor V deficiency in an elderly hemodialysis patient: a case report and literature review

  • 期刊论文

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  • 发表日期：

    2026年2月7日

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  • Ayaka Hane,
  • Yuriko Yasuda,
  • Yuri Matsuda,
  • Naoki Kachi,
  • Shunsuke Kitamura,
  • Yosuke Shibata,
  • Katsuhiko Morimoto

摘要

Background

Acquired factor V deficiency (AFVD) is a rare hemorrhagic disorder caused by autoantibodies against coagulation factor V. Clinical manifestations vary widely, ranging from asymptomatic cases to severe hemorrhage or even thrombosis, and diagnosis is often challenging due to its rarity and nonspecific presentation. Reported triggers include bovine thrombin, antibiotics, surgery, autoimmune diseases, infections, and malignancy, although up to 20% of cases are idiopathic. We report a case of AFVD in an elderly hemodialysis patient, in whom corticosteroid therapy was effective but ultimately complicated by fatal gastrointestinal bleeding.

Case presentation

An 87-year-old woman on hemodialysis for diabetic nephropathy was admitted with acute cholecystitis and treated with ceftriaxone. On day 16, she developed bleeding from a gastrostomy site with markedly prolonged prothrombin time (69.3 s) and activated partial thromboplastin time (260.4 s). Cross-mixing studies showed inhibitor patterns, and factor V activity was reduced to 1.3%, with an inhibitor titer of 33.3 Bethesda units/mL, confirming AFVD. Despite no bleeding from vascular access sites, she developed progressive anemia and gingival bleeding. Intravenous prednisolone (40 mg/day) was initiated, resulting in normalization of coagulation parameters and recovery of factor V activity to 111.7% within 30 days. However, on day 57, she developed massive melena. Endoscopy revealed hemorrhagic duodenal ulcers with exposed vessels, requiring endoscopic hemostasis. Recurrent gastrointestinal bleeding occurred on day 61, and she died of hemorrhagic shock.

Conclusions

This case illustrates the diagnostic process and therapeutic challenges of AFVD in a hemodialysis patient. Corticosteroid therapy was effective in eradicating the inhibitor and restoring factor V activity, but fatal gastrointestinal bleeding occurred as a severe treatment-related complication. Clinicians should be aware of AFVD as a rare but important cause of acquired coagulopathy, and careful monitoring for corticosteroid-associated adverse effects is crucial, especially in elderly and critically ill individuals.