{
  "abstract": "Introduction: Acquired hemophilia A (AHA) is a rare autoimmune hemorrhagic disorder characterized by neutralizing autoantibodies against coagulation factor VIII (FVIII). First-line immunosuppressive therapy with corticosteroids with or without rituximab achieves complete remission (CR) in 60-80% of patients; however, relapse occurs in approximately 15-33% of cases after treatment cessation. Management of relapsed AHA remains challenging, with limited evidence for optimal second-line approaches. Bruton’s tyrosine kinase (BTK) inhibitors have emerged as promising agents for antibody-mediated autoimmune diseases.\n\nCase description: Herein, we report the first case of relapsed AHA successfully treated with orelabrutinib monotherapy after relapse following an initial response to a conventional corticosteroid-rituximab regimen. A 45-year-old male with type 1 diabetes mellitus and hyperthyroidism presented with spontaneous bleeding and was diagnosed with AHA associated with underlying autoimmune endocrinopathies. He achieved complete remission (CR) following prednisone and rituximab but relapsed 3 months after treatment cessation. Orelabrutinib 50 mg orally once daily was initiated as an experimental off-label approach. The patient demonstrated progressive laboratory improvement, achieving a second CR approximately 2 months after orelabrutinib initiation, with undetectable FVIII inhibitor and normalized FVIII activity. Sustained on-treatment remission was maintained throughout 5 months of therapy without bleeding episodes or adverse events, and remission has been maintained after completion of the planned 6-month course and subsequent treatment discontinuation.\n\nConclusion: This case supports the therapeutic potential of selective BTK inhibition as a targeted, steroid-sparing strategy in relapsed AHA and highlights the need for prospective clinical trials to validate this approach.",
  "abstract_shown": true,
  "affiliations": [
    "Department of Hematology, The Second Hospital of Hebei Medical University, Hebei Key Laboratory of Hematology",
    "Special Examination Department, The Second Hospital of Hebei Medical University"
  ],
  "article_type": null,
  "arxiv": null,
  "author_total": 6,
  "authors": [
    {
      "affs": [
        0
      ],
      "name": "Yujie Guo"
    },
    {
      "affs": [
        1
      ],
      "name": "Feifei Zhang"
    },
    {
      "affs": [
        0
      ],
      "name": "Yabei Zuo"
    },
    {
      "affs": [
        0
      ],
      "name": "Yan Wang"
    },
    {
      "affs": [
        0
      ],
      "name": "Zhiyun Niu"
    },
    {
      "affs": [
        0
      ],
      "name": "Jingyu Zhang"
    }
  ],
  "bibtex": "https://research.draconitic.com/paper/cxjmj243hkp5t.bib",
  "cited_by": 0,
  "doi": "10.3389/fimmu.2026.1931049",
  "fulltext_url": null,
  "id": "cxjmj243hkp5t",
  "issns": [
    "1664-3224"
  ],
  "landing_url": "https://www.frontiersin.org/articles/10.3389/fimmu.2026.1931049/full",
  "language": null,
  "licence": "CC-BY-4.0",
  "licence_label": "CC BY 4.0",
  "licence_source": "article",
  "licence_url": "https://creativecommons.org/licenses/by/4.0/",
  "metadata_licence": null,
  "native_subjects": [
    [
      "openalex",
      "2720|Hemophilia Treatment and Research"
    ]
  ],
  "openalex": "W7224079175",
  "pdf_url": "https://www.frontiersin.org/journals/immunology/articles/10.3389/fimmu.2026.1931049/pdf",
  "pmcid": null,
  "pmid": null,
  "published": "2026-10-28",
  "publisher": "Frontiers Media SA",
  "ris": "https://research.draconitic.com/paper/cxjmj243hkp5t.ris",
  "sources": [
    {
      "first_seen": "2026-10-30T12:19:17Z",
      "native_id": "10.3389/fimmu.2026.1931049",
      "source": "crossref",
      "url": "https://www.frontiersin.org/articles/10.3389/fimmu.2026.1931049/full"
    }
  ],
  "subjects": [
    {
      "field": "biology",
      "origin": "issn:1664-3224",
      "subfield": "immunology"
    }
  ],
  "title": "Case Report: Relapsed acquired hemophilia A following prednisone-rituximab therapy successfully treated with orelabrutinib",
  "title_text": "Case Report: Relapsed acquired hemophilia A following prednisone-rituximab therapy successfully treated with orelabrutinib",
  "type": "peer-reviewed",
  "updated_at": "2026-10-30T12:19:17Z",
  "url": "https://research.draconitic.com/paper/cxjmj243hkp5t",
  "venue": "Frontiers in Immunology",
  "versions": []
}