Case Report: Relapsed acquired hemophilia A following prednisone-rituximab therapy successfully treated with orelabrutinib
- 1Department of Hematology, The Second Hospital of Hebei Medical University, Hebei Key Laboratory of Hematology
- 2Special Examination Department, The Second Hospital of Hebei Medical University
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.
Case 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.
Conclusion: 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.
Related
- Rituximab-Induced Crohn’s Disease-like Colitis Following Treatment for Post-transplant Lymphoproliferative Disorder in Chronic Active Epstein-Barr Virus Infection: A Case Report
- Clinical features of ROHHAD syndrome and treatment outcomes following rituximab therapy: a descriptive case series of Chinese children
- Generative AI Successfully Captures Visual Mental Imagery
- Case Report: Fulminant aggressive NK-cell leukemia with hemophagocytic lymphohistiocytosis and multiple organ dysfunction successfully bridged to allogeneic hematopoietic stem cell transplantation
- Modeling DDAVP response from endothelial storage to individualized dosing in mild hemophilia A and symptomatic carriers
BibTeX
@article{guo2026case,
title = {{Case Report: Relapsed acquired hemophilia A following prednisone-rituximab therapy successfully treated with orelabrutinib}},
author = {Yujie Guo and Feifei Zhang and Yabei Zuo and Yan Wang and Zhiyun Niu and Jingyu Zhang},
journal = {Frontiers in Immunology},
year = {2026},
month = oct,
doi = {10.3389/fimmu.2026.1931049},
url = {https://www.frontiersin.org/articles/10.3389/fimmu.2026.1931049/full},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - Case Report: Relapsed acquired hemophilia A following prednisone-rituximab therapy successfully treated with orelabrutinib
AU - Yujie Guo
AU - Feifei Zhang
AU - Yabei Zuo
AU - Yan Wang
AU - Zhiyun Niu
AU - Jingyu Zhang
PY - 2026
DA - 2026/10/28
JO - Frontiers in Immunology
DO - 10.3389/fimmu.2026.1931049
UR - https://www.frontiersin.org/articles/10.3389/fimmu.2026.1931049/full
AB - 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. Case 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. Conclusion: 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.
SN - 1664-3224
ER -