Coagulation Factor Deficiency, Hemophilia, Hemophilia A, Hemophilia B, Liver Cirrhosis, Liver Failure, Liver Transplant Disorder
Conditions
Keywords
liver transplant, hemophilia, coagulation, thrombosis, bleeding, cirrhosis
Brief summary
The goal of this observational study is to learn about the changes in coagulation factor VIII and IX levels in patients undergoing liver transplantation to help guide future management of coagulation factor replacement in patients with hemophilia and liver disease. The question we aim to answer is: should the recommendations for factor replacement in patients with hereditary bleeding disorders be altered in the setting of end stage liver cirrhosis? Participants will be asked to provide two blood samples, one at the beginning of their liver transplant, and one after their liver transplant.
Detailed description
Current guidelines for management of hemophilia B suggest replacement of factor IX to 100% prior to major abdominal surgery. However, in patients with concurrent liver cirrhosis where the liver does not produce Factor IX, is it worth considering adjusting the factor replacement strategy? We recently had a case of a patient with Hemophilia B and end-stage liver disease (ESLD) who underwent orthotopic liver transplantation and received the standard pre-operative recombinant factor IX replacement. His case was complicated by intra-cardiac thrombus and hypotension. We conduct a small study to assess the pre-operative thromboelastography (TEG) and factor levels in ESLD patient which we hope will help guide clinical decision making in future hemophilia B patients with cirrhosis.
Interventions
None listed
Sponsors
Stanford University
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* participants who have end stage liver disease who are listed for liver transplantation and have an accepted organ offer * age \> 18+ * MELD \> 25
Exclusion criteria
* undergoing multi-organ transplant * tumor MELD exception points * has hereditary coagulation disease * currently on therapeutic blood thinner or anti-platelet medication (ie. aspirin, plavix, warfarin, heparin)
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Factor VIII level | 12 hours | We will collect Factor VIII level pre- and post- transplant |
| Factor IX level | 12 hours | We will collect Factor IX level pre- and post- transplant |
| Thromboelastography (TEG) values | 12 hours | We will collect thromboelastography values pre- and post- transplant |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Complications | 24 hours | We will collect data on bleeding or clotting events during the liver transplant surgery and 24 hours post-operatively |
Countries
United States
Contacts
Primary ContactAlexandra Ruan, MD
Outcome results
None listed