What does this research mean for the field?

Acquired hemophilia A can rarely manifest as life-threatening hemorrhagic pericardial effusion and tamponade, which can be successfully managed with prompt pericardiocentesis, hemostatic agents, and immunosuppression. Novelty: ClaimNovelty.INCREMENTAL. Consensus alignment: ConsensusAlignment.NEUTRAL.

What question did this study set out to answer?

This case highlights the presentation and management of pericardial tamponade due to factor VIII inhibitors in a patient with acquired hemophilia A.

May 20, 2026

C51-28 A Bleeding Heart: Factor VIII Inhibitor-Induced Pericardial Tamponade

Key Points

This case highlights the presentation and management of pericardial tamponade due to factor VIII inhibitors in a patient with acquired hemophilia A.
75-year-old male with a history of COPD and atrial fibrillation presented with hypotension and dyspnea.
Diagnosis confirmed via echocardiogram and coagulation studies including prolonged aPTT and low FVIII activity.
Treatment involved emergent pericardiocentesis, fresh frozen plasma, IVIg, and recombinant activated factor VII.
Emergent pericardiocentesis drained 710 mL of bloody fluid, leading to improved hemodynamic status.
Initial labs showed long aPTT (50.1 sec), low FVIII activity (14%), and FVIII inhibitor titer of 7.8 Bethesda units.
Repeat echocardiograms confirmed resolution of effusion after treatment.

Abstract

Abstract Introduction Acquired hemophilia A (AHA) is a rare condition caused by autoantibodies against factor VIII (FVIII), leading to severe bleeding. While spontaneous hemorrhage is common, pericardial effusion and tamponade due to FVIII inhibitors are exceptionally rare. This case highlights a patient with FVIII inhibitor-induced pericardial effusion progressing to tamponade and obstructive shock, emphasizing diagnostic work-up and incidence. Case Presentation A 75-year-old male with a history of COPD, ulcerative colitis, atrial fibrillation, and remote pacemaker placement presented with hypotension and dyspnea. Physical exam revealed hypotension (BP 60/30 mmHg) and muffled heart sounds. Echocardiogram confirmed a large pericardial effusion with tamponade physiology. Emergent pericardiocentesis drained 710 mL of frank bloody fluid, improving the shock state.Lab findings showed prolonged aPTT (50.1 sec), which failed to correct on mixing study, low FVIII activity (14%), and a FVIII inhibitor titer of 7.8 Bethesda units. Hematology consultation agreed with the diagnosis of AHA. Initial management included fresh frozen plasma and DDAVP while awaiting further confirmatory labs, followed by IVIg, methylprednisolone, and recombinant activated factor VII (rFVIIa) for hemostasis. The patient stabilized with recombinant FVIII and immunosuppressive therapy. Repeat echocardiography confirmed resolution of effusion. Discussion AHA has an incidence of 1.5 cases per million per year, mostly affecting older adults. While soft tissue and mucosal bleeding are typical, hemorrhagic pericardial effusion and tamponade are exceedingly rare.Diagnosis relies on prolonged aPTT uncorrected by mixing studies, low FVIII activity, and elevated FVIII inhibitor titers. Management includes hemostatic agents (e.g., rFVIIa, activated prothrombin complex concentrates) and immunosuppression.In this case, hemorrhagic pericardial effusion directly led to tamponade and obstructive shock, highlighting the need for early recognition. Prompt pericardiocentesis, coagulation studies, and targeted treatment were essential for stabilization. Conclusions This case underscores the rare but life-threatening manifestation of FVIII inhibitor-induced pericardial effusion and tamponade. Clinicians must consider AHA in patients with unexplained hemorrhagic effusions and prolonged aPTT to enable timely intervention. This abstract is funded by: N/a

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Cite This Study

Graumann et al. (Fri,) studied this question.

synapsesocial.com/papers/6a0d4fd2f03e14405aa9b4d8 https://doi.org/https://doi.org/10.1093/ajrccm/aamag162.1525

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