Got Band-aids? A Case Study about Hemophilia by Amy Albright

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Got Band-aids?A Case Study about
HemophiliabyAmy Albright

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Patient History

• 82 year old female
• History of complications including known
  Hepatitis A, B, and C
• Last year she developed a progressive bleed after
  a gastric biopsy was performed

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History - continued

• Around the same time, she underwent a partial
  gastrectomy with resulting bleeding
• While in the hospital, further bleeding occurred
  from the abdominal incision, vagina, mouth, and
  IV site

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Questions to Consider?

• 1.) What tests were performed on this patient?
• 2.) What was the cause of this womans
• bleeding problem?
• 3.) What was done to correct the problem?

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Test Performed
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Mixing Study
Patient plasma is mixed with normal plasma to
observe the resulting clotting times.
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Mixing Study Results
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Mixing Study Interpretation

• Progressive inhibitor , indicating an inhibitor
  to a specific factor

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Results

• Factor Assay lt 1

• Bestheda Titer 22.0
• (1 BU amount of inhibitor that neutralizes 50
  of the FVIII in NPP)

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Diagnosis

• Acquired severe factor VIII deficiency due to
  Factor VIII inhibitor
• Acquired Hemophilia

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Understanding Acquired Hemophilia

• This is a form of hemophilia which develops in
  patients who were previously unaffected
• These patients develop an antibody against a
  specific clotting factor. This antibody is
  called an acquired factor inhibitor.

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Understanding Acquired Hemophilia - continued

• Very rare affects 1 in a million people
• Risks of developing it
• - pregnancy
• - autoimmune diseases
• - cancer
• - may emerge in the elderly w/o any risk
  factors

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Symptoms

• Much like that of this 82 year old woman
• spontaneous bleeding
• bleeding from surgical incisions
• bleeding from puncture sites which may stay
  bleeding until treated

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Initial Treatment

• Initially given fresh frozen plasma (FFP) and
  packed red blood cells (PRBCs)
• 10 units of Cryoprecipitate
• Noted the bleeding wasnt stopping
• Lab results of inhibitor came back

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Treatment once the diagnosis is made includes.

• Reducing inhibitor production
• - giving bolus dose of the clotting factor
  being affected
• - giving excess factor to overcome
  bleeding problem to obtain hemostasis
• Supplying patient with blood products
  to replace blood cells that were lost

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Treatment - continued

• Gave bolus of Factor VIII (neutralize the
  inhibitor) followed by excess FVIII to maintain
  hemostasis
• Patient was still bleeding
• Indicates large amount of inhibitor

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Treatment - continued

• Discussed plasma pheresis followed by FVIII
  infusion as a last resort
• Gave recombinant Factor VIIa
• Factor VIIa stopped the bleeding
• Continued the F VIII regimen to lower the
  inhibitor titer

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Other Infusions

• Packed RBCs
• Platelets
• Continuous F VIIa therapy every 12 hours
• (half life of FVII is about 5-6 hours)

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Patient Outcome

• Two weeks past initial bleeding Bestheda titer
  2.6 due to neutralization of the FVIII inhibitor
• Bleeding episodes subsided
• Continuous monitoring and supportive therapy
• continuous monitoring of PTT
• factor VIII infusion when necessary
• work to suppress the immune system

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Patient Outcome - continued

• Immune system suppression achieved via
• Steroid immune system suppressant (Cytoxan)
  medications
• High dose intravenous immunoglobulin

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Case Summary

• 82 y.o. patient presented with uncontrollable
  bleeding post-op
• Various coagulation studies performed
• Diagnosed with Acquired Hemophilia
• Treatment with Factor VIIa
• Continue to monitor

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References

• 1.) Ledford-Kraemer. All mixed up about mixing
  studies. 2004. http//www.clot-ed.com
• 2.) Karim, R., Retzinger, G., Acquired
  Hemophilia. Lab Lines. 2004 vol 10, issue 2.
• 3.) http//www.med.umich.edu

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Credits

• This case study was
• prepared by
• Amy Albright, MT(ASCP)
• while she was a Medical Technology student in
• the 2004 MT Class at William Beaumont
• Hospital, Royal Oak, MI.