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Interactive Case Study of a Patient with Severe Hemophilia A - Experience from Slovenia

24. 3. 2021

Assoc. Prof. Dr. Irena Preložnik Zupan (University Medical Centre Ljubljana) describes an interactive case study of a patient with severe hemophilia A and progressive cataracts in both eyes.

Personal History

  • 55-year-old man with severe hemophilia A, no history of FVIII inhibitors.
  • Secondary prophylaxis with SHL rFVIII (Refacto AF) 30 IU/kg twice weekly.
  • Comorbidities include mild anemia, cured hepatitis C, and advanced arthropathy of several joints.
  • Total knee arthroplasty in both knees in 2018 and 2019.
  • No reported spontaneous or traumatic bleeding in 2019.
  • Prophylaxis with emicizumab (Hemlibra) started in February 2020 at the patient's request due to poor venous access.
  • 44 weeks on emicizumab prophylaxis without any resolved spontaneous or traumatic bleeding.

Current Condition

  • Severe hemophilia A well-controlled with emicizumab 3 mg/kg subcutaneously every 14 days.
  • Cataract in the left eye requiring surgical intervention.
  • Not currently on any other medication.

Objective Findings

  • 55-year-old man with normotension and severe hemophilia A without inhibitors.
  • Mature cataract in the left eye, early-stage cataract in the right eye.
  • Normal intraocular pressure.
  • Visual acuity before surgery: left eye 0.3; right eye 0.6.
  • Topical anesthesia planned for the procedure on the left eye.

Laboratory Tests

  • Mild normocytic anemia, otherwise normal blood count and biochemistry without significant pathologies.
  • FVIII activity level 0.005 (chromogenic assay, bovine reagents).
  • FVIII activity level 0.20 (chromogenic assay, human reagents).
  • No confirmed FVIII inhibitors (chromogenic Bethesda assay, bovine reagents).

 

How should postoperative care be managed for this patient? (check the correct statement)

#64

Please review the text section of the case study above and vote on the course of action you would take before watching the video.



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