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Semin Thromb Hemost
DOI: 10.1055/a-2788-1642
Original Article

Emicizumab in Acquired Hemophilia A: A Real-World Case Series with Patient-Level Outcome Analysis

Authors

  • Ilenia Calcaterra    *

    1   Department of Clinical Medicine and Surgery, Regional Reference Centre for Coagulation Disorders, Federico II University, Naples, Italy

  • Carmine De Luca   *

    1   Department of Clinical Medicine and Surgery, Regional Reference Centre for Coagulation Disorders, Federico II University, Naples, Italy

  • Guido D'Errico

    1   Department of Clinical Medicine and Surgery, Regional Reference Centre for Coagulation Disorders, Federico II University, Naples, Italy

  • Ciro Miele

    1   Department of Clinical Medicine and Surgery, Regional Reference Centre for Coagulation Disorders, Federico II University, Naples, Italy

  • Chiara Caputo

    1   Department of Clinical Medicine and Surgery, Regional Reference Centre for Coagulation Disorders, Federico II University, Naples, Italy

  • Raffaele Russo

    1   Department of Clinical Medicine and Surgery, Regional Reference Centre for Coagulation Disorders, Federico II University, Naples, Italy

  • Paolo Conca

    1   Department of Clinical Medicine and Surgery, Regional Reference Centre for Coagulation Disorders, Federico II University, Naples, Italy

  • Ernesto Cimino

    1   Department of Clinical Medicine and Surgery, Regional Reference Centre for Coagulation Disorders, Federico II University, Naples, Italy

  • Anna Guida

    2   Centre for Congenital Haemorrhagic Disorders and Thrombotic Diseases, Rheumatologic and Immunologic Clinic, San Giovanni di Dio and Ruggi D'Aragona University Hospital, Salerno, Italy

  • Antonella Tufano   *

    1   Department of Clinical Medicine and Surgery, Regional Reference Centre for Coagulation Disorders, Federico II University, Naples, Italy

  • Matteo Di Minno   *

    1   Department of Clinical Medicine and Surgery, Regional Reference Centre for Coagulation Disorders, Federico II University, Naples, Italy
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Abstract

Acquired Hemophilia A (AHA) is a rare bleeding disorder caused by factor VIII inhibitors. Standard therapies are limited by thrombotic risk and prolonged hospitalization. Emicizumab, approved for congenital Hemophilia A, has emerged as a potential alternative in AHA based on case reports and early clinical trial data. To evaluate the efficacy and safety of Emicizumab in AHA through a retrospective real-world case series and a systematic literature review with patient-level data analysis. We retrospectively analyzed five AHA cases treated with Emicizumab at two Italian centers and performed a PRISMA-compliant systematic review of published reports, extracting and analyzing patient-level data using Joanna Briggs Institute tools. In the real-world cohort, early Emicizumab use in five patients with high-titer inhibitors and severe bleeding led to rapid hemorrhagic control, early withdrawal of bypassing agents, and no thrombotic or adverse events. All five patients received immunosuppression, and inhibitor eradication was achieved in 60% of patients, but for 40% follow up is still ongoing. The literature review identified 24 patients from 18 publications. Early Emicizumab administration (at admission) was associated with reduced bleeding recurrence (0% vs. 56.3%), shorter in-hospital stay (median 23.5 days vs. 39 days), and lower bleeding-related mortality (0% vs. 12.5%) compared with delayed administration. Early Emicizumab initiation appears to be a safe and effective strategy for AHA management, particularly in fragile or high-risk populations. Its subcutaneous route, favorable safety profile, and ability to reduce hospitalization support its integration into first-line therapeutic algorithms. Further prospective studies are warranted to define.

Keywords

acquired hemophilia A - Emicizumab - bleeding control - hospitalization - comorbidities - patient-level data

Authors' Contributions

I.C., C.D.L., A.T., and M.D.M.: conception and design of the study. A.G., C.C., E.C., G.D.E., P.C., and R.R.: acquisition of data. I.C. and C.D.L. analysis and interpretation of data. I.C., C.D.L., and C.M.: drafting the article. A.T. and M.D.M.: revising it critically for important intellectual content. All authors approved the final version to be submitted.


Declaration of GenAI Use

During the preparation of this article, the authors used ChatGPT (OpenAI, GPT-4o, July 2025) to assist in improving the language, structure, and clarity of the text. The AI was used exclusively for language polishing and editorial refinement. No content was generated without human supervision. All outputs were carefully reviewed, edited, and validated by the authors, who take full responsibility for the integrity and accuracy of the final article.


* These authors contributed equally to this article.


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Publication History

Received: 28 August 2025

Accepted: 13 January 2026

Accepted Manuscript online:
14 January 2026

Article published online:
28 January 2026

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