Case 374 -- A male in his 30s scheduled for Liver transplant

Contributed by Franklin Sedarat MD MSc, Lirong Qu MD PhD, Darrell J. Triulzi MD
Published on line in January 2004


PATIENT HISTORY:

The patient was a male in his 30s with history of severe hemophilia A and end-stage liver disease secondary to hepatitis C. The course of his disease was complicated by progressive hepatic encephalopathy. The patient was scheduled for Liver transplant. Blood bank was contacted for the dosing of factor VIII perioperatively. The patient did not produce inhibitors to factor VIII.

There were two issues related to this case: 1) Factor VIII dosing during the transplantation procedure, and 2) the production of factor VIII by the transplanted liver.

ANSWERS TO PATIENT'S ISSUES:

The patient had been receiving 4000 units of recombinant FVIII daily for one week prior to transplantation for minor bleeding. On the day of surgery, 4000u (100%) was given before the start of surgery and 2000 to 4000 units given q2h or q4h during the surgery. The level and frequency of dosing was based on bleeding during the operation.

Post-operative dosing: 0-hour: 4000u, 8-hour: 4000u, then 2000u q8-hour for next 48 hours. A factor VIII level should be obtained after 4 to 6 doses for defining additional doses.

In this case factor VIII:C level was 0.38 (normal range 0.60-1.50 U/mL) before surgery and was totally corrected to 1.44 three days after surgery. The dosing of factor VIII concentrate and patient's coag profile are listed in Table 1. Liver function tests are shown in Table 2. Note the correction of PT and PTT after transplant. The maintanance of corrected coagulation parameters after day 3 was due to production of coagulation factors by the transplanted liver.

In one study by Wilde J. Et al, in a UK single-centre, Hemophilia patients were followed after liver transplant. These patients had liver failure due to chronic hepatitis C (HCV) infection. Between March 1990 and March 2001, 11 patients (mean age 46 years: nine hemophilia A, two hemophilia B) underwent transplantation. In this study factor concentrate replacement was administered using a continuous infusion regimen following initial bolus dosing. Concentrate infusion was discontinued at a median of 36 h (range 24-72 h) post transplant.

DISCUSSION


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