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Case Report Open Access

Immunoglobulin G4‑related acquired hemophilia: A case report

  • Authors:
    • Xiaoyan Li
    • Wei Duan
    • Xiang Zhu
    • Jianying Xu
  • View Affiliations / Copyright

    Affiliations: Department of Respiratory Medicine, Shanxi DAYI Hospital of Shanxi Medical University, Taiyuan, Shanxi 030032, P.R. China, Department of Pathology, Peking University Third Hospital, Beijing 100191, P.R. China
    Copyright: © Li et al. This is an open access article distributed under the terms of Creative Commons Attribution License.
  • Pages: 3988-3992
    |
    Published online on: November 14, 2016
       https://doi.org/10.3892/etm.2016.3898
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Abstract

Acquired hemophilia A (AHA) is a relatively rare and life‑threatening bleeding disorder whose pathogenesis is not completely understood. The present study reports a rare case of immunogubulin (IgG)4‑related AHA with multisystemic involvement. A 55‑year old male patient presented with symptoms of bronchial asthma and multiple subdermal hematomas. Chest computed tomography showed multiple diffuse nodular lesions with thickening of bronchovascular bundles, and scattered high‑density spots in both lung lobes. Laboratory investigations showed increased activated partial prothrombin time (120.0 sec), a markedly decreased factor VIII (FVIII) activity (0.5%), a high‑titer of FVIII inhibitor (27.2 Bethesda units/ml) and a marked increase in serum IgG4 (>4.03 g/l) level. Left inguinal lymph node biopsy revealed capsular thickening with marked lymphoplasmacytic infiltration, occlusive phlebitis and irregular fibrosis. Immunostaining revealed numerous IgG4‑positive plasma cells (>100 cells/human plasma fibronectin) in the nodular lesions, with an IgG4/IgG ratio of >40%. The symptoms were markedly alleviated following corticosteroid therapy. The current study presents the first reported case of a rare IgG4‑related AHA that presented with unusual clinical features and multisystemic involvement. The patient responded well to corticosteroid therapy. Documentation of such rare cases will help in characterizing the pathogenesis, and prompt recognition and timely treatment of this rare disorder.
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Copy and paste a formatted citation
Spandidos Publications style
Li X, Duan W, Zhu X and Xu J: Immunoglobulin G4‑related acquired hemophilia: A case report. Exp Ther Med 12: 3988-3992, 2016.
APA
Li, X., Duan, W., Zhu, X., & Xu, J. (2016). Immunoglobulin G4‑related acquired hemophilia: A case report. Experimental and Therapeutic Medicine, 12, 3988-3992. https://doi.org/10.3892/etm.2016.3898
MLA
Li, X., Duan, W., Zhu, X., Xu, J."Immunoglobulin G4‑related acquired hemophilia: A case report". Experimental and Therapeutic Medicine 12.6 (2016): 3988-3992.
Chicago
Li, X., Duan, W., Zhu, X., Xu, J."Immunoglobulin G4‑related acquired hemophilia: A case report". Experimental and Therapeutic Medicine 12, no. 6 (2016): 3988-3992. https://doi.org/10.3892/etm.2016.3898
Copy and paste a formatted citation
x
Spandidos Publications style
Li X, Duan W, Zhu X and Xu J: Immunoglobulin G4‑related acquired hemophilia: A case report. Exp Ther Med 12: 3988-3992, 2016.
APA
Li, X., Duan, W., Zhu, X., & Xu, J. (2016). Immunoglobulin G4‑related acquired hemophilia: A case report. Experimental and Therapeutic Medicine, 12, 3988-3992. https://doi.org/10.3892/etm.2016.3898
MLA
Li, X., Duan, W., Zhu, X., Xu, J."Immunoglobulin G4‑related acquired hemophilia: A case report". Experimental and Therapeutic Medicine 12.6 (2016): 3988-3992.
Chicago
Li, X., Duan, W., Zhu, X., Xu, J."Immunoglobulin G4‑related acquired hemophilia: A case report". Experimental and Therapeutic Medicine 12, no. 6 (2016): 3988-3992. https://doi.org/10.3892/etm.2016.3898
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