15. Acquired FX Deficiency

Acquired FX Deficiency

  • Reported by Korsan Bengsten (1962) in association with 1° amyloidosis (AL)
    - Most common factor deficiency in 1° amyloidosis; rarely occurs in 2° amyloidosis
    - FX is adsorbed onto amyloid fibrils
    - Resolves with therapy of underlying disease
  • Liver disease, vitamin K deficiency
  • Malignancies
    - Spindle cell thymoma, renal/adrenal/gastric ca, AML
  • Drugs (sodium valproate)
  • Inhibitors: Mycoplasma pneumoniae, topical thrombin
    - Treated with steroids, IVIG, plasmapheresis