Transcript Amy Albright Medical Technology Student William Beaumont

Got Band-aids?
A Case Study about Hemophilia
by
Amy Albright
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?
Test Performed
PT
normal
PTT
abnormally
Mixing Study-Inhibitor
Screen
DRVVT
abnormal
Factor Assay
abnormal
Bestheda Titer
abnormal
normal
Mixing Study
Patient plasma is mixed with normal plasma to
observe the resulting clotting times.
Mixing Study Results
Normal Plasma
29.6
Patient Plasma
106.9
Normal Plasma + Patient
Plasma (1:1) immediate
53.1
Normal Plasma + Patient
Plasma (1:1) incubated
72.0
Mixing Study
Interpretation
• Progressive inhibitor , indicating an
inhibitor to a specific factor
Results
• Factor Assay= < 1%
• Bestheda Titer = 22.0
(1 BU= amount of inhibitor that neutralizes 50% of the
FVIII in NPP)
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.