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

1. Got Band-aids?A Case Study about HemophiliabyAmy Albright

2. 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

3. 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

4. Questions to Consider? 1.) What tests were performed on this patient? 2.) What was the cause of this woman’s bleeding problem? 3.) What was done to correct the problem?

5. Test Performed

6. Mixing Study Patient plasma is mixed with normal plasma to observe the resulting clotting times.

7. Mixing Study Results

8. Mixing Study Interpretation • Progressive inhibitor , indicating an inhibitor to a specific factor

9. Bestheda Titer = 22.0 (1 BU= amount of inhibitor that neutralizes 50% of the FVIII in NPP) Results • Factor Assay= < 1%

10. Diagnosis • Acquired severe factor VIII deficiency due to Factor VIII inhibitor Acquired Hemophilia

11. 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.

12. 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

13. 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

14. Initial Treatment • Initially given fresh frozen plasma (FFP) and packed red blood cells (PRBC’s) • 10 units of Cryoprecipitate • Noted the bleeding wasn’t stopping • Lab results of inhibitor came back

15. 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

16. 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

17. 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

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

19. 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

20. Patient Outcome - continued… • Immune system suppression achieved via: • Steroid + immune system suppressant (Cytoxan) medications • High dose intravenous immunoglobulin

21. 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

22. 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

23. 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.