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[Acquired haemophilia A]

R E Brouwer1, M V Huisman, J D Feuth

  • 1Afd. Algemene Interne Geneeskunde, Academisch Ziekenhuis, Leiden.

Nederlands Tijdschrift Voor Geneeskunde
|July 5, 1997
PubMed
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This case study highlights a severe bleeding disorder caused by an acquired Factor VIII inhibitor in a 74-year-old man. Prompt diagnosis and treatment, including immunosuppression, are crucial for managing this rare condition.

Area of Science:

  • Hematology
  • Internal Medicine

Background:

  • Acquired Factor VIII inhibitors cause severe bleeding disorders, often linked to pregnancy, malignancy, or autoimmune conditions.
  • Acquired hemophilia A presents with a significant hemorrhagic diathesis that can be life-threatening.

Observation:

  • A 74-year-old male presented with a severe bleeding disorder.
  • Coagulation tests revealed prolonged APTT, normal PT, low F VIII, and high F VIII inhibitor levels (8.8 BU).

Findings:

  • The patient's bleeding disorder was attributed to an acquired F VIII inhibitor.
  • Treatment options include factor concentrates (human/porcine F VIII, FEIBA, rFVIIa) and immunosuppressive therapy.

Implications:

  • Prompt diagnosis via coagulation assays and medical history is crucial.

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  • Immunosuppressive therapy can lead to inhibitor eradication in over 60% of patients.
  • Management requires specialist consultation for severe hemorrhagic diathesis.