Management of Kidney Transplantation in a Factor VII-Deficient Patient: Case Report

Transplantation in patients with congenital bleeding disorders is apioneering challenge requiring an integrated approach involving dif-ferent specialists. Renal transplantation is the most frequent cause ofsolid organ transplant. It is rarely performed in individuals with con-genital haemorrhagic disorders, with only few reports in haemophiliaA patients. We performed renal transplantation in a 53 year-old manwith congenital coagulation factor VII (FVII) deficiency and endstage renal disease. FVII activity ranged between 4% and 8%. Bleed-ing history consisted only in prolonged bleeding at accidental cuts. Genotyping of FVII gene revealed a compound heterozygosity forthe Met298Ile aminoacid substitution in the catalytic domain(g10811a transition in exon 8) and the IVS7+5g/a change (FVII Laz-io). Peri-operative bleeding was prevented by the administration ofrecombinant activated Factor VII (rFVIIa). Treatment schedule wasbased on rFVIIa administration at the dosage of 30 mcg/kg givenbefore surgery, after fifteen minutes, then after 1 and 4 h. After theintervention, administration was continued every 6 h for the first7 days, then twice a day for the following seven days. FVII basalactivity prior surgery was 5%, with an INR of 2.5. During intra-operative period and the following days of treatment INR rangedfrom 1.0 to 1.8 and FVII activity never dropped under 20%. Intra-operative bleeding was normal (< 300 mL) in absence of any bleed-ing or thrombotic complication. To our knowledge this is the firstreported case of kidney transplantation in a FVII-deficient patient.Our report confirms the feasibility and the safety of rFVIIa in majorsurgical procedures at potential high risk of thrombosis like solidorgan transplant. Careful evaluation of administration doses andtherapeutic schedules as well as a multidisciplinary approach arerequired.