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Acquired haemophilia A with a recalcitrant high-titre factor VIII inhibitor in the setting of interstitial lung disease
  1. Lova Sun,
  2. David B Sykes
  1. Department of Hematology, Massachusetts General Hospital, Boston, Massachusetts, USA
  1. Correspondence to Dr David B Sykes, dbsykes{at}


Acquired haemophilia A (AHA) is a bleeding disorder that results from autoantibodies against factor VIII (FVIII). A 70-year-old man with a history of interstitial lung disease presented with spontaneous bleeding into his thigh. He had undetectable FVIII levels and a high-titre FVIII inhibitor (>2000 Bethesda units/mL) and was diagnosed with AHA. He had several relapses, required multiple haemostatic and immunosuppressive treatments but eventually achieved a stable remission after 2 years of therapy.

Our patient matches the typical elderly male demographic of AHA. His relapsing course with remarkably high and persistent inhibitor titre highlights the need for close monitoring and aggressive upfront treatment. Whereas cyclophosphamide and steroids are often used first line in AHA, rituximab has also shown efficacy in refractory patients with high inhibitor levels. The FVIII and inhibitor concentration on presentation have been associated with treatment response and may be used as prognostic factors to tailor immunosuppressive regimens.

  • Haematology (incl Blood Transfusion)
  • Haematology (drugs And Medicines)

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  • Contributors LS and DBS researched and drafted the entirety of the manuscript.

  • Competing interests None declared.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.