43 y.o. male with JAK-2 mutation developed cirrhosis due to Budd-Chiari syndrome and had extensive portal and mesenteric vein thrombosis that precluded a liver transplant alone. His liver disease was complicated by refractory ascites, encephalopathy, severe muscle wasting and MELD-Na of 36. In addition, he developed hepatorenal syndrome and hemodialysis was initiated. Despite lack of intestinal failure, the patient required a multivisceral graft for anatomical reasons of lack of ability to restore portal inflow to the liver graft.

Multivisceral transplant (MVT) including liver, pancreas, stomach, intestine and partial colon was performed followed by kidney transplant the following day. Despite preoperative partial arterial embolization of the native superior mesenteric and splenic arteries, he required massive transfusion of 47 units PRBC’s due to incomplete embolization and severe portal hypertension. The patient received Thymoglobulin induction and has been on triple drug immunosuppression (tacrolimus, mycophenolate mofetil, prednisone).

Thrombophilia was managed with IV heparin perioperatively and enoxaparin was started postoperatiely. This was associated with gastrointestinal bleed from the gastrojejunal anastomosis during the first postoperative week. The heparin dose reduction during the period of GI bleed was associated with the development of non-occlusive thrombi in both internal jugular veins. In addition, he was treated with hydroxyurea for thrombocytosis, subsequently plateletpheresis and anagralide when platelet count reached >1.4 million. By the time of discharge, the patient was on full enteral nutrition and had normal renal function.

This case highlights the indication of MVT for patients with hypercoaguable states and extensive portomesenteric thrombosis. In this patient, his hypercoaguable state was more extensive than many due to involvement of his hepatic veins leading to Budd Chiari syndrome. The cause of his hypercoaguable state was known to be a JAK-2 mutation, although the cause is not always identifiable. Perioperative anticoagulation with standard medications prevented thrombotic complications, however thrombocytosis developed (likely from splenectomy) and was not controlled by hydroxyurea and required the use of plateletpheresis and anagralide. Our plan will be for life-long anticoagulation and we anticipate switching from enoxaparin to apixaban after discontinuation of protocol monthly bowel biopsies and ostomy closure.