(P-TA-6) Management of Therapeutic Plasma Exchange in Factor V Leiden Thrombophilia with Recurrent Thromboembolism on Long-Term Anticoagulant therapy: A Case Report

Factor V Leiden (FVL) is an inherited thrombophilia that leads to a hypercoagulable state and risk of venous thromboembolism. Patients having FVL mutation with recurrent thromboembolism require long-term anticoagulation. For such patients on anticoagulant therapy who need therapeutic plasma exchange (TPE), there are no standard guidelines about the choice of replacement fluid, the appropriate anticoagulant or withholding anticoagulant before TPE to prevent hemostatic complications. Data about the safety of TPE with regards to bleeding or thrombotic complications in patients with FVL mutation is limited.

Study

Design/Methods:  A 36-year-old male with a history of Denys-Drash syndrome, testicular cancer, end-stage renal disease due to focal segmental glomerulosclerosis status post dual renal transplantation at ages 11 and 13 due to Wilms tumor, melena pending biopsy, and heterozygous FVL mutation with recurrent deep venous thrombosis, status post inferior vena cava filter placement, developed antibody mediated rejection of his second renal transplantation. A plan of 7-9 TPE procedures was devised. Patient was on warfarin with the INR therapeutic range of 2.0 to 3.0.  A ramp-up approach was followed and TPE began with half (0.5) plasma volume (PV) exchange that was gradually increased in the subsequent procedures to full (1.0) PV exchange, with close monitoring of coagulation parameters.

Results/Findings:

Renal biopsy was performed 3 days before the 1st TPE. Since warfarin was withheld 4 days before the biopsy, INR before the 1st TPE was 1.3. INR before the 2nd TPE resulted critically high at > 9.5. Although pre-analytic heparin contamination of sample from the central venous line could not be excluded, repeat INR by point of care testing about 8 hours later was 4.2. Patient did not have bleeding and was advised to hold warfarin at night. Pre-procedure INR of the subsequent procedures, though not critical, had frequent fluctuations and required warfarin dose adjustments (Table 1). Patient underwent 9 TPE procedures using 5% albumin as the replacement fluid, on alternate days, each TPE followed by IVIG infusion. Plasma was not used in any TPE. The initial 4 procedures involved 0.5 PV, the next 2 involved 0.75 PV exchange, and the final 3 had full (1.0) PV exchange. After the 6th TPE, patient was switched to subcutaneous heparin. He did not have any major bleeding complication during the TPE series but continued to have melena and biopsy revealed colon adenocarcinoma. He required 2 RBCs transfusions during the series for safe extracorporeal volume during TPE.

Conclusions:

Managing TPE in patients with FVL on warfarin therapy is challenging. A ramp-up approach of TPE and close monitoring of coagulation parameters with warfarin dose adjustment or substitution to subcutaneous heparin could be a viable strategy. Further studies need to be performed to prevent hemostatic complications in such patients.