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Background <p>Acquired hemophilia A (AHA) is a rare hemorrhagic disorder caused by autoantibodies against coagulation factor VIII (FVIII). AHA during hemodialysis (HD) initiation is uncommon, but dialysis catheterization and intradialytic anticoagulation may increase bleeding risk. Ocular bleeding is also an uncommon, potentially vision-threatening manifestation. We report a case of AHA unmasked by acute vitreous hemorrhage with marked intraocular pressure elevation during HD initiation.</p> Case presentation <p>A 61-year-old male with hypertension, diabetes mellitus, and proliferative diabetic retinopathy who had discontinued medical follow-up was transferred to our hospital with ocular pain, visual loss, severe hypertension, and renal failure. Ophthalmologic evaluation showed no light perception in the right eye, markedly elevated intraocular pressure to 78&#xa0;mmHg, and a flat anterior chamber, findings consistent with vitreous hemorrhage and secondary ocular hypertension in the setting of proliferative diabetic retinopathy and impaired hemostasis. Antihypertensive treatment was initiated, and a temporary dialysis catheter was placed for emergency renal replacement therapy. On day 2, isolated prolongation of activated partial thromboplastin time (APTT) and persistent bleeding from the catheter insertion site were observed. Detailed investigations revealed markedly reduced FVIII activity and the presence of an FVIII inhibitor. APTT cross-mixing tests demonstrated a time-dependent inhibitor pattern, leading to the diagnosis of AHA. HD was initially performed without anticoagulants under bypassing hemostatic therapy. Prednisolone (PSL) was started on day 5, a tunneled cuffed catheter was placed on day 19, and reduced-dose oral cyclophosphamide (CPA) was added on day 28. Subsequently, the bleeding tendency improved, APTT normalized, and the FVIII inhibitor titer decreased. The patient continued maintenance HD while immunosuppression was tapered and was discharged on day 210.</p> Conclusions <p>This case highlights acute vitreous hemorrhage with marked intraocular pressure elevation as an unusual initial clue to AHA during HD initiation. When ocular hemorrhage is accompanied by unexplained isolated APTT prolongation or persistent catheter-site bleeding, AHA should be promptly considered, even in patients with diabetic retinopathy or uremic bleeding risk. Safe management requires early hemostatic treatment, immunosuppression, and individualized coordination of ophthalmologic care, vascular access, and dialysis anticoagulation.</p>

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Acquired hemophilia A unmasked by vitreous hemorrhage at hemodialysis initiation: a case report and literature review

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年7月3日

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  • Takaya Suzuki,
  • Kai Matsuo,
  • Yukino Yokokawa,
  • Masahiro Miyata,
  • Takaaki Nawano,
  • Eri Matsuki,
  • Sayumi Watanabe,
  • Keita Kamei,
  • Kazunobu Ichikawa,
  • Masafumi Watanabe,
  • Akane Yamada

摘要

Background

Acquired hemophilia A (AHA) is a rare hemorrhagic disorder caused by autoantibodies against coagulation factor VIII (FVIII). AHA during hemodialysis (HD) initiation is uncommon, but dialysis catheterization and intradialytic anticoagulation may increase bleeding risk. Ocular bleeding is also an uncommon, potentially vision-threatening manifestation. We report a case of AHA unmasked by acute vitreous hemorrhage with marked intraocular pressure elevation during HD initiation.

Case presentation

A 61-year-old male with hypertension, diabetes mellitus, and proliferative diabetic retinopathy who had discontinued medical follow-up was transferred to our hospital with ocular pain, visual loss, severe hypertension, and renal failure. Ophthalmologic evaluation showed no light perception in the right eye, markedly elevated intraocular pressure to 78 mmHg, and a flat anterior chamber, findings consistent with vitreous hemorrhage and secondary ocular hypertension in the setting of proliferative diabetic retinopathy and impaired hemostasis. Antihypertensive treatment was initiated, and a temporary dialysis catheter was placed for emergency renal replacement therapy. On day 2, isolated prolongation of activated partial thromboplastin time (APTT) and persistent bleeding from the catheter insertion site were observed. Detailed investigations revealed markedly reduced FVIII activity and the presence of an FVIII inhibitor. APTT cross-mixing tests demonstrated a time-dependent inhibitor pattern, leading to the diagnosis of AHA. HD was initially performed without anticoagulants under bypassing hemostatic therapy. Prednisolone (PSL) was started on day 5, a tunneled cuffed catheter was placed on day 19, and reduced-dose oral cyclophosphamide (CPA) was added on day 28. Subsequently, the bleeding tendency improved, APTT normalized, and the FVIII inhibitor titer decreased. The patient continued maintenance HD while immunosuppression was tapered and was discharged on day 210.

Conclusions

This case highlights acute vitreous hemorrhage with marked intraocular pressure elevation as an unusual initial clue to AHA during HD initiation. When ocular hemorrhage is accompanied by unexplained isolated APTT prolongation or persistent catheter-site bleeding, AHA should be promptly considered, even in patients with diabetic retinopathy or uremic bleeding risk. Safe management requires early hemostatic treatment, immunosuppression, and individualized coordination of ophthalmologic care, vascular access, and dialysis anticoagulation.