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
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
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?
Mixing Study Patient plasma is mixed with normal plasma to observe the resulting clotting times.
Mixing Study Interpretation • Progressive inhibitor , indicating an inhibitor to a specific factor
Bestheda Titer = 22.0 (1 BU= amount of inhibitor that neutralizes 50% of the FVIII in NPP) Results • Factor Assay= < 1%
Diagnosis • Acquired severe factor VIII deficiency due to Factor VIII inhibitor Acquired Hemophilia
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.
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
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
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
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
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
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
Other Infusions: • Packed RBCs • Platelets • Continuous F VIIa therapy every 12 hours (half life of FVII is about 5-6 hours)
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
Patient Outcome - continued… • Immune system suppression achieved via: • Steroid + immune system suppressant (Cytoxan) medications • High dose intravenous immunoglobulin
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
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
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.