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International Journal of Hematology

, Volume 108, Issue 3, pp 335–338 | Cite as

Acquired hemophilia A associated with autoimmune pancreatitis with serum IgG4 elevation

  • Taisuke Narazaki
  • Shojiro Haji
  • Yasuhiro Nakashima
  • Yasuhiro Tsukamoto
  • Mariko Tsuda
  • Akiko Takamatsu
  • Hirofumi Ohno
  • Takamitsu Matsushima
  • Tomoko Matsumoto
  • Keiji Nogami
  • Midori Shima
  • Motoaki Shiratsuchi
  • Yoshihiro Ogawa
Case Report
  • 186 Downloads

Abstract

A case of acquired hemophilia A (AHA) that developed in a patient with autoimmune pancreatitis (AIP) is presented. A 64-year-old woman was diagnosed with AIP in 2007. The symptoms resolved with prednisolone (PSL). Although the dose of PSL was tapered to 7.5 mg/day for maintenance, serum IgG4 levels remained high. She suddenly presented with subcutaneous bleeding in 2015. Her activated partial thromboplastin time was prolonged (80.0 s). A mixing test showed an inhibitor pattern, factor VIII (FVIII) activity was less than 1%, and FVIII inhibitor was 290 BU/mL. She was diagnosed with AHA. Her serum IgG4 was elevated to 133 mg/dL. She was treated first with PSL alone, but she developed bladder tamponade. Cyclophosphamide and activated prothrombin complex concentrate were combined with PSL. She then achieved hemostasis, and FVIII inhibitor disappeared. FVIII inhibitor had been detected since PSL was tapered and AHA recurred two months later. An enzyme-linked immunosorbent assay showed that the inhibitor was mainly IgG4 and IgG1. This case suggests that elevation of IgG4 may be associated with the development of both AHA and AIP.

Keywords

Acquired hemophilia A Autoimmune pancreatitis IgG4 

Notes

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

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Copyright information

© The Japanese Society of Hematology 2018

Authors and Affiliations

  • Taisuke Narazaki
    • 1
  • Shojiro Haji
    • 1
  • Yasuhiro Nakashima
    • 1
  • Yasuhiro Tsukamoto
    • 1
  • Mariko Tsuda
    • 1
  • Akiko Takamatsu
    • 1
  • Hirofumi Ohno
    • 1
  • Takamitsu Matsushima
    • 1
  • Tomoko Matsumoto
    • 2
  • Keiji Nogami
    • 2
  • Midori Shima
    • 2
  • Motoaki Shiratsuchi
    • 1
  • Yoshihiro Ogawa
    • 1
    • 3
  1. 1.Department of Medicine and Bioregulatory Science, Graduate School of Medical SciencesKyushu UniversityFukuokaJapan
  2. 2.Department of PediatricsNara Medical UniversityKashiharaJapan
  3. 3.Department of Molecular and Cellular Metabolism, Graduate School of Medical and Dental SciencesTokyo Medical and Dental UniversityTokyoJapan

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