Factor XII deficiency acquired by orthotopic liver transplantation

Status: 
Ready to upload
Record number: 
1497
Adverse Occurrence type: 
MPHO Type: 
Estimated frequency: 
Rare
Time to detection: 
2 years
Alerting signals, symptoms, evidence of occurrence: 
Prolonged activated partial thromboplastin time (aPTT) with no bleeding noted when investigated for renal biopsy two years after orthotopic liver transplant. Liver transplant donor had prolonged aPTT at the time of her death.
Demonstration of imputability or root cause: 
Prior to transplant, recipient had normal aPTT and some abnormal coagulation factors attributed to liver failure. Liver donor had prolonged aPTT at the time of transplant with no significant bleeding. Post transplant recipient had no significant bleeding diathesis and prolonged aPTT was noted two years post transplant as work up for renal biopsy. Post transplant Factor XII activity was <2 in the recipient. No pre-transplant or donor Factor XII activity was available.
Imputability grade: 
2 Probable
Groups audience: 
Suggest new keywords: 
Liver Transplant
Factor XII deficiency
Acquired Deficiency
Reference attachment: 
Suggest references: 
Osborn, NK, et al, 2006. Factor XII deficiency acquired by orthotopic liver transplantation: case report and review of the literature. Am J Transplant 6:1743-5.
Expert comments for publication: 
Factor XII initiates the contact activation reactions and intrinsic blood coagulation in vitro. Severe factor XII deficiency is a rare disorder that is inherited as an autosomal recessive trait and affected individuals have prolonged aPTT with no bleeding diathesis. Thus in this case after liver transplant, this was identified two years later. Cases of other factor deficiencies transmitted by liver transplantation have been reported.