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Acquired Factor VIII Deficiency: A Case Presentation

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Isabel Gutierrez

on 20 November 2014

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Transcript of Acquired Factor VIII Deficiency: A Case Presentation

Back to DC
DC is a 42-year old female with CLL and weighs 59.3kg. DC had an acute drop in her Hgb was was given 2 units of PRBCs on 11/08/14. It was noted that she had ascitic hemorrhage. She was moved to the CV-ICU on 11/09/14. She was given 2 units of FFP and Vitamin K there. DC was diagnosed to have Acquired Hemophilia A.
What next?
She was given one dose of her chemotherapy on 11/10/14. DC was started on Kcentra on 11/11/14. She was no longer severely bleeding after the Kcentra, but she was still bleeding from her PICC site.
Recap Summary
Treatment Options:
Acute Bleeding
Bypassing Agents
Kcentra
Novoseven RT*
FEIBA VH
Increase circulating Factor VIII
Obizur*
Desmopressin
Clotting Cascade
Acquired Hemophilia A:
A Case Presentation

Treatment Options:
Inhibitor Eradication
Immunosuppressive Agents
SoluMedrol/Cytoxan
Targeted/biologic Therapy
Rituxan
Special Populations
Cyclosporine
Azathioprine
IVIG
Immunoadsorption
Immune Tolerance
Diagnosis Requirements
High APTT not corrected by mixing study
Low Factor VIII levels with presence of inhibitors
By: Isabel Gutierrez
PharmD Candidate 2015
Preceptor: Bill Braun, PharmD

Acquired Hemophilia A
rare bleeding disorder
autoantibodies of clotting Factor VIII
Picture retrieved from - http://oregonstate.edu/instruct/bb450/fall14/stryer7/10/figure_10_26.jpg
Picture retrieved from - https://www.hemophilia.org/sites/default/files/basic-page/images/rare-bleeding-and-clotting-disorders-resource-room.jpg
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Acquired vs. Congenital Hemophilia
Diagnosis for patient with no previous history
High activated partial thromboplastin time (APTT) that is unable to be corrected with a mixing study
Low Factor VIII level with evidence of inhibitor activity
Diagnosis
DC is a 42 year old African American female with a diagnosis of chronic lymphocytic leukemia. She was first admitted on 10/24/14 for abdominal pain that had been going on for about a week. On 11/02/14 she was started on sulfasalazine by the GI doctor for enteritis with jejunal and ileal inflammation. DC was transferred to the ICU on 11/07/14 for an acute drop in her Hgb to 3.9 and bleeding from her PICC line. After transfusion with 2 units of PRBCs, her Hgb improved to 9.2.
Patient Case: DC
APTT was 159.1!
AND
Factor VIII level was 0.1!
* FDA approved for Acquired Hemophilia
She was given 3 doses of 5,000 mcg NovoSeven RT every 2 hours with a modest response. After speaking with Pharmacy, Heme/Onc decided to try Kcentra 3,000units every 8 hours PRN.
With the chemotherapy, DC was given 1g of SoluMedrol and 1,300mg of Cytoxan. The next day on 11/11/14 she was given 650mg Rituxan.
Acute Bleeds
1st line - Bypassing Agents
New Factor VIII medications
Inhibitor Eradication
Steroids/cytotoxic therapy
Rituximab
Picture retrieved from - http://bossip.files.wordpress.com/2012/02/middle-aged-woman.jpg
Thank you for coming!
Any Questions?

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References
Franchini, Massimo, and Giuseppe Lippi. "Acquired Factor VIII
Inhibitors." Blood 2nd ser. 112 (2008): 250-55. Blood. American Society of Hematology. Web. 10 Nov. 2014

Grethlein, MD, Sara J, and Craig M Kessler, MD, MACP.
"Acquired Hemophilia Medication." Acquired Hemophilia Medication. Medscape, 10 Nov. 2014. Web. 17 Nov. 2014.

Grethlein, MD, Sara J, and Craig M Kessler, MD, MACP.
"Acquired Hemophilia Treatment." Acquired Hemophilia Treatment. Medscape, 10 Nov. 2014. Web. 17 Nov. 2014.

Clinical Pharmacology. Available at <www.clinicalpharmacology.com>.

Micromedex. Available at <www.micromedexsolutions.com>.
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Full transcript